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Dermatomyositis classically has an inflammatory rash associated with a suppurative polymyositis. The rash may precede the development of the muscular involvment, be concurrent, or occur after. Cases without muscle involvement are called dermatomyositis sine myositis. The disease can occur at any age or sex. The rash has a violaceous scaly appearance occurring on the face, shoulders, and extensor surfaces of the forearms and thighs. A heliotrope rash and Gottron's papules may be present. Poikiloderma is frequent. Photosensitivity may be present.

This is an autoimmune disorder. It is associated with anti-nuclear antibodies including anti-Jo-1 antibody. There is a sparse lichenoid inflammatory cell infiltrate composed of HLA-DR positive macrophages and CD4 positive T lymphocytes. The membrane attack complex (MAC) has been demonstrated by immunofluorescence along the dermoepidermal junction and blood vessels. The lupus band test is usually negative.

Interestingly, a dermatomyositis-like syndrome has been associated with toxoplasmosis, pregnancy, hydroxyurea, and penicillamine therapy.


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Number, characteristics, and classification of patients with dermatomyositis seen by dermatology and rheumatology departments at a large tertiary medical center.

University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA.


J Am Acad Dermatol. 2007 Dec;57(6):937-43. Abstract quote

BACKGROUND: The current diagnostic criteria for dermatomyositis (DM) exclude patients without muscle involvement. As a result there is a paucity of research related to the complete spectrum of the disease.

OBJECTIVE: The goal of this study was to evaluate differences in the clinical manifestations of DM seen by dermatology relative to rheumatology. We hypothesized that patients with minimal (hypomyopathic) or no (amyopathic) muscle disease would more likely be seen in dermatology, whereas those with more severe (classic) muscle disease would be seen in rheumatology.

METHODS: We performed a retrospective chart review of patients with DM seen by our dermatology and rheumatology departments to classify spectrum, presentation, and complications. Patients seen between July 1, 2003, and June 30, 2006, were identified by Current Procedural Terminology billing code 710.3. Patients with mixed connective tissue diseases or miscoded DM were excluded.

RESULTS: In all, 131 (65%) patients seen in dermatology, 58 (29%) in rheumatology, and 13 (6%) in both departments were identified. In all, 83 (69%) patients seen in dermatology, 27 (23%) in rheumatology, and 10 (8%) in both departments met criteria for inclusion in the study. The number of patients seen in rheumatology given the classification of classic DM (CDM) (24 of 27 [89%]), hypomyopathic DM (2 of 27 [7%]), and amyopathic DM (ADM) (1 of 27 [4%]) differed significantly from dermatology, where CDM comprised 27 of 83 (33%), hypomyopathic DM comprised 23 of 83 (28%), and ADM comprised 33 of 83 (40%) of the population, respectively (P < .001). Sex, ethnicity, and rates of interstitial lung disease differed between departments. There was no difference in the rates of interstitial lung disease between CDM and ADM (P = .30). The degree of muscle involvement did not correlate with the rates of DM-associated malignancy (P = .57). Few patients with ADM had muscle biopsy (n = 1) or electromyography (n = 7) testing. Positive anti-Jo-1 was seen in 2 of 96 patients (2%; one CDM and one ADM, both with interstitial lung disease), reflecting an overall low prevalence of this autoantibody, or a potential problem with the laboratory assay.

LIMITATIONS: Patients reflect the population in only one institution and, thus, the results may not be generalizable to other settings or referral centers. Because this is a retrospective chart review, results are limited by missing data and nonstandardized physical examinations and laboratory data across patients and physicians.

CONCLUSIONS: There is a clear difference in DM presentation to dermatology and rheumatology by degree of myositis-complicated disease.


Mi2, an auto-antigen for dermatomyositis, is an ATP-dependent nucleosome remodeling factor.

Wang HB, Zhang Y.

Department of Biochemistry and Biophysics, Curriculum in Genetics and Molecular Biology, Lineberger Comprehensive Cancer Center, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599-7295, USA.

Nucleic Acids Res 2001 Jun 15;29(12):2517-21 Abstract quote

Dynamic changes in chromatin structure play an important role in transcription regulation. Recent studies have revealed two mechanisms that alter chromatin structure. One involves ATP-dependent chromatin remodeling, and the other involves acetylation of the core histone tails.

We have previously purified and characterized a multi-subunit protein complex, NuRD, which possesses both nucleosome remodeling and histone deacetylase activities. Despite extensive biochemical characterization of the complex, little is known about the functions of its individual components. In this study, we focused on Mi2, a component of the NuRD complex. We found that, similar to the native NuRD complex, recombinant Mi2 is a DNA-dependent, nucleosome-stimulated ATPase. Kinetic analysis of the ATP hydrolysis reaction indicated that the differential stimulation of the Mi2 ATPase by DNA and nucleosomes were primarily due to their differential effects on the turnover number of the reaction. Furthermore, we demonstrated that recombinant Mi2 is an efficient nucleosome remodeling factor when compared to that of the native NuRD complex.

Our results define the biochemical function of Mi2 and set the stage for understanding the mechanism of nucleosome remodeling in a defined reconstituted system.

Possible role for Parvovirus B19 J Cutan Pathol 2000;27:505-515
Seven prospectively studied patients with connective tissue disease like symptomatology had evidence of B19 infection

Severe perturbations of the blood T cell repertoire in polymyositis, but not dermatomyositis patients.

Benveniste O, Cherin P, Maisonobe T, Merat R, Chosidow O, Mouthon L, Guillevin L, Flahault A, Burland MC, Klatzmann D, Herson S, Boyer O.

Laboratoire de Biologie et Therapeutique des Pathologies Immunitaires, Service de Medecine Interne, and Laboratoire de Neuropathologie, Hopital Pitie-Salpetriere, Paris, France.

J Immunol 2001 Sep 15;167(6):3521-9 Abstract quote

Polymyositis and dermatomyositis are diseases characterized by muscle weakness and muscle inflammatory infiltrates. Their pathogenesis remains unclear. A central role for endomysial autoaggressive CD8(+) T cells is suspected in polymyositis and for perivascular B cells in dermatomyositis.

We compared the T cell repertoire of 10 polymyositis and 10 dermatomyositis patients by immunoscope, a method providing a global assessment of the T cell repertoire and a sensitive detection of clonal T cell expansions. Samples were analyzed qualitatively and quantitatively in the blood (unsorted cells and CD4(+) and CD8(+) cells) and in muscle infiltrates. Dramatic perturbations of the T cell repertoire were observed in the blood of polymyositis but not dermatomyositis patients (p < 0.0005), the latter being undistinguishable from controls. These perturbations were due to oligoclonal expansions of CD8(+) T cells and most blood clonal expansions were also found in muscle.

These results indicate that the pathogenesis of polymyositis and dermatomyositis is different and reinforce the view that polymyositis but not dermatomyositis is an autoimmune CD8(+) T cell-mediated disease. Moreover, this method may be helpful for the differential diagnosis of polymyositis and dermatomyositis and for noninvasive follow-up of polymyositis patients.


MALIGNANCY 10-15% of cases
Often associated with subepidermal bullous disease

Dermatomyositis and malignancy in Tunisia: a multicenter national retrospective study of 20 cases.

Mebazaa A, Boussen H, Nouira R, Rokbani L, Ben Osman-Dhahri A, Bouaouina N, Laouani-Kechrid C, Louzir B, Zahaf A, Kamoun MR.

Department of Dermatology, La Rabta University Hospital, Tunis.


J Am Acad Dermatol 2003 Apr;48(4):530-4 Abstract quote

OBJECTIVE AND METHODS: The aim of our study was to report the epidemiologic, clinical, and biologic profiles of dermatomyositis (DM) associated with malignancy in patients from Tunisia. From January 1982 to January 2000, we collected retrospectively 20 case reports of DM associated with cancer from the different university hospital centers of Tunisia. Initial workup included anamnesis, clinical examination, cancer staging and classification, serum muscle enzymes (creatine phosphokinase, lactate dehydrogenase, aldolase, and transaminases), electromyography, and muscular biopsy. We calculated the median survival and mean value of all the variables. Comparisons of statistical tests were done with the Kruskal-Wallis test.

RESULTS: Among the 130 DM cases of our study, 20 were associated with cancer (15.38%). The mean age of our patients was 49.6 years and the sex ratio (female/male) was equal to 3. Cancers were mainly those of the breast (35%) and nasopharynx (25%). DM followed a paraneoplastic course in 90% of the cases. The profile of seric muscular enzymes showed a significant statistical difference (P =.05) between a group of patients with severe muscular weakness and a group with moderate muscle weakness only for creatine kinase. The median survival was 36.5 months after diagnosis of DM and 48.6 months after that of cancer. The 5-year actuarial survival was 38% as related to cancer and 16% as related to DM. Mortality was 45%, in 90% as a result of cancer.

CONCLUSIONS: In our study, nasopharyngeal carcinoma represents the second cancer associated with DM, after breast neoplasm, demonstrating the frequency of these 2 cancers in our country. Despite our reduced number of study samples, our study also suggests a relationship between severe muscle weakness and high seric muscle enzymes.

Dermatomyositis and
sarcoid-like reaction associated with multiple myeloma treated effectively by high-dose chemotherapy and autologous peripheral blood stem cell transplantation.

Chakraverty R, Rabin N, Peggs K, Robinson S,
Duncan JR, Yong K.

Institute of Cancer Studies, University of Birmingham, Edgbaston, UK.

Bone Marrow Transplant 2001 Jun;27(11):1215-7 Abstract quote

We report the case of a 46-year-old male who developed dermatomyositis and a sarcoid-like reaction in association with testicular relapse of multiple myeloma.

The myositis progressed despite chemotherapy directed at the underlying malignant disorder and immunosuppressive treatment. There was, however, a dramatic and sustained response to high-dose chemotherapy and autologous peripheral blood stem cell transplantation which resulted in resolution of the myopathy and partial resolution of the sarcoid-like reaction.

This case report highlights the potential of autologous stem cell transplantation as treatment for para-neoplastic disorders associated with haematological malignancies.

Primary lung cancer associated with polymyositis/
dermatomyositis, with a review of the literature.

Fujita J, Tokuda M, Bandoh S, Yang Y, Fukunaga Y, Hojo S, Ueda Y, Dobashi N, Dohmoto K, Ishida T, Takahara J.

First Department of Internal Medicine, Kagawa Medical University, Japan.

Rheumatol Int 2001 Feb;20(2):81-4 Abstract quote

It has been suggested that lung cancer is frequently associated with polymyositis/dermatomyositis (PM/DM). The purpose of this study was to describe the clinical features of primary lung cancer associated with PM/DM.

We first describe the clinical features of two cases treated in our hospital, and then provide a review of the literature. Finally, 24 patients (five females and 19 males) with primary lung cancer associated with PM/DM are retrospectively evaluated. Histological types of lung cancer were as follows: small cell lung cancer (n = 7), squamous cell carcinoma (n = 5), adenocarcinoma (n = 2), others (n = 5), and unknown (4). The onset of PM/DM is frequently observed before the detection of lung cancer.

This is the first report to describe the clinical features of lung cancer associated with PM/DM.

Routine vs extensive malignancy search for adult dermatomyositis and polymyositis: a study of 40 patients.

Sparsa A, Liozon E, Herrmann F, Ly K, Lebrun V, Soria P, Loustaud-Ratti V, Bouyssou-Gauthier ML, Boulinguez S, Bedane C, Jauberteau MO, Vidal E, Bonnetblanc JM.

Department of Dermatology, University Hospital of Limoges, Limoges, France.


Arch Dermatol 2002 Jul;138(7):885-90 Abstract quote

OBJECTIVE: To identify potential risk factors and the yield of routine screens for early detection of malignancy associated with dermatomyositis (DM) and polymyositis (PM).

DESIGN: Retrospective study of malignancies in all patients with DM or PM followed up between the years 1981 and 2000 and a review of the relationship of DM and PM to malignancy, the usefulness of various tests or examinations for malignancy search, and the patients' course.

SETTING: Departments of internal medicine and dermatology in a teaching hospital.

PATIENTS: Forty consecutive adult patients with DM (33 cases) or PM (7 cases).

MAIN OUTCOME MEASURES: (1) Rate of false-negative results of routine workup and yield (percentage of positive results) of blind malignancy search and (2) comparison of 16 characteristics in patients with malignancy vs those without.

RESULTS: Malignancy occurred in 16 patients: 13 with DM and 3 with PM. In all cases, the diagnosis of malignancy was made concurrently with or shortly after the diagnosis of DM or PM. Factors associated with malignancy were recruitment in the internal medicine department (P =.02), constitutional symptoms (P<.01), a rapid onset of DM or PM (P =.02), the lack of Raynaud phenomenon (P<.01), and a higher mean erythrocyte sedimentation rate (P<.01) and creatine kinase level (P<.01). Initial routine search failed to discover 4 malignancies, 3 of which were discovered at an advanced stage by more extensive investigations. The positive result yield of blind malignancy search was only 13% (11 of 87), but reached 28% (5 of 18) for blind abdominal-pelvic and thoracic computed tomographic scans.

CONCLUSION: Extensive search for malignancy, particularly computed tomographic scans, may be warranted in at least a subset of patients with DM or PM and risk factors of malignancy.

Dermatomyositis associated with malignant melanoma--a marker of poor prognosis?

Schiller M, Bohm M, Hensen P, Riemann H, Luger TA, Nashan D.

Department of Dermatology, University of Munster, Munster, Germany
J Am Acad Dermatol. 2006 Feb;54(2):221-6. Abstract quote  

BACKGROUND: Dermatomyositis (DM) is an inflammatory connective tissue disorder well recognized as a paraneoplastic syndrome in adults.

OBJECTIVE: The objective of this study was to assess the prognosis of DM associated with malignant melanoma (MM).

PATIENTS AND METHODS: We systematically searched databases (PubMed, MEDLINE, and WEB OF SCIENCE) for articles reporting the concurrence of DM and MM. For the literature study, time of onset of DM in relation to diagnosis of MM (before, concomitant with, or after), stage of MM after restaging (according to the American Joint Committee on Cancer [AJCC] guidelines, 2001), and survival time after diagnosis of DM were recorded. Survival time studies and univariate statistical analyses were performed. Furthermore, we present our own clinical case of a patient with DM concomitantly occurring with regional lymph node metastasis of MM.

RESULTS: In 5 cases DM occurred before, in 6 cases concomitantly with, and in 6 cases after progression of MM. Univariate analysis identified the AJCC stage of MM as a significant prognostic factor. Gender, age, and the time interval between onset of DM and progression of melanoma were unrelated. The 1-year actuarial survival rate was 0% for patients with DM when occurring with MM at stage IV and 60% when occurring with MM at stage III (P < .05). The estimated mean survival time was 6.6 months for patients with MM stage IV and 57 months for stage III.

LIMITATIONS: The conclusions from this study are limited by the relatively small number of articles that reported the association of MM and DM.

CONCLUSION: DM occurring in patients with MM at stage IV is connected with an extremely poor prognosis, whereas the few reported patients with DM and MM at stage III, including our case, have a prognosis similar to stage III patients without DM.

Scleromyxedema (lichen myxedematosus) associated with dermatomyositis.

Launay D, Hatron PY, Delaporte E, Hachulla E, Devulder B, Piette F.

Department of Internal Medicine, Claude Huriez Hospital, 1, place de Verdun, 59037 Lille Cedex. France.

Br J Dermatol 2001 Feb;144(2):359-62 Abstract quote

A 41-year-old white man is described with papules of the lower and upper back, the neck and the upper chest, a marked deposition of mucin in the upper reticular dermis, and an IgG lambda monoclonal gammopathy strongly evocative of scleromyxedema (lichen myxedematosus). Additionally, he developed intense myalgia, muscle weakness and rhabdomyolysis, which were associated with heliotrope erythema, photosensitivity and an erythematous rash of the dorsum of the hands with Gottron's papules. Muscle biopsy revealed an inflammatory myositis, and dermatomyositis was diagnosed.

The association of dermatomyositis and secondary mucinosis, or muscle involvement in primary papular mucinosis are not rare. However, the association between scleromyxedema and dermatomyositis has only exceptionally been reported.

Terbinafine-induced dermatomyositis: a case report and literature review of drug-induced dermatomyositis.

Department of Pathology and Laboratory Medicine, Weill Medical College of Cornell University, New York, NY, USA.


J Cutan Pathol. 2008 Jan;35(1):74-81. Abstract quote

Dermatomyositis, a connective tissue disease syndrome where antibodies to the endothelium of the microvasculature of the skin, muscle and lung are implicated in lesional propagation, is characterized by photodistributed erythema, heliotrope rash, Gottron's papules, muscle weakness and interstitial pulmonary fibrosis. Endotheliotropic viruses and underlying neoplasia are among the inciting triggers. Uncommon drugs, namely the lipid-lowering agents, have been implicated in dermatomyositis.

The patient, a 57-year-old man, developed a photodistributed rash and muscle weakness following treatment with the antifungal medication, terbinafine. A skin biopsy was performed, showing an atrophying interface dermatitis with pandermal mucinosis and striking vasculopathic changes including endothelial cell necrosis with denudement and basement membrane zone reduplication. Ultrastructural studies confirmed the presence of endothelial cell injury.

Direct immunofluorescent testing showed prominent staining of C5b-9 along the dermal-epidermal junction and within the vasculature. Western blot studies showed strong seroreactivity of his serum to an endothelial-based protein weighing 45,000, a common target described in other microvascular injury-based syndromes.

We have shown a temporal association between use of terbinafine and the development of dermatomyositis. The exact basis remains speculative. One potential hypothesis is based on the fact that terbinafine, the active agent in terbinafine, triggers apoptosis of human endothelial cells in culture. Enhanced endothelial cell apoptosis results in the displacement of various cellular antigens creating a state of neoantigenicity; its attendant sequelae is held to be one of anti-endothelial cell antibody formation, a defining pathogenetic event in the evolution of dermatomyositis. The second may be because of the effects of the drug on the promotion of an interferon-rich T-helper-1-dominant cytokine milieu.

Central nervous system vasculitis as a complication of refractory dermatomyositis.

Regan M, Haque U, Pomper M, Pardo C, Stone J.

Department of Medicine, Johns Hopkins University Vasculitis Center, Baltimore, Maryland 21205, USA.

J Rheumatol 2001 Jan;28(1):207-11 Abstract quote

We describe a 47-year-old woman with refractory dermatomyositis (DM) who developed progressive cognitive dysfunction. Magnetic resonance imaging showed a large cerebral infarction, and the diagnosis of central nervous system (CNS) vasculitis was confirmed by both angiogram and brain biopsy.

Her DM and CNS vasculitis responded promptly to the institution of daily cyclophosphamide, and her previously refractory disease entered remission.



MR imaging in amyopathic dermatomyositis.

Lam WW, Chan H, Chan YL, Fung JW, So NM, Metreweli C.

Department of Diagnostic Radiology and Organ Imaging, Chinese University of Hong Kong, Shatin, Hong Kong.

Acta Radiol 1999 Jan;40(1):69-72 Abstract quote

PURPOSE: Amyopathic dermatomyositis is a distinct clinical entity with cutaneous involvement but no myopathy. We conducted a prospective study to investigate the role of MR imaging in these patients.

MATERIAL AND METHODS: Out of 40 Chinese patients presenting with dermatomyositis, based on clinical assessment and normal serum muscle enzymes 10 were diagnosed as having amyopathic dermatomyositis. These 10 patients underwent MR imaging for evaluation of any subclinical muscle involvement.

RESULTS: Three patients demonstrated abnormal signal intensity in muscles on both T2- and fat suppression sequences. Thus, one-third of patients with dermatomyositis and clinically normal muscles may have detectable muscle inflammation on MR images, indicating that MR has a potential role for locating the relevant biopsy site and for longitudinal follow up. Six of the 10 patients had malignant disease diagnosed before or after diagnosis of the cutaneous manifestation. Nasopharyngeal carcinoma was the most common malignant disease in this group of patients.

CONCLUSION: MR imaging is recommended for demonstrating subclinical muscle involvement in patients with the clinical diagnosis of amyopathic dermatomyositis. We also recommend screening for malignancy, particularly nasopharyngeal carcinoma, in Southern Chinese patients with dermatomyositis.


Clinical Correlations With Dermatomyositis-Specific Autoantibodies in Adult Japanese Patients With Dermatomyositis: A Multicenter Cross-sectional Study.

Hamaguchi Y, Kuwana M, Hoshino K, Hasegawa M, Kaji K, Matsushita T, Komura K, Nakamura M, Kodera M, Suga N, Higashi A, Ogusu K, Tsutsui K, Furusaki A, Tanabe H, Sasaoka S, Muro Y, Yoshikawa M, Ishiguro N, Ayano M, Muroi E, Fujikawa K, Umeda Y, Kawase M, Mabuchi E, Asano Y, Sodemoto K, Seishima M, Yamada H, Sato S, Takehara K, Fujimoto M.
Department of Dermatology, Kanazawa University Graduate School of Medical Science, 13-1 Takaramachi, Kanazawa, Ishikawa 920-8641, Japan. mfujimoto@derma.m.kanazawa-u.ac.jp.

Arch Dermatol. 2011 Apr;147(4):391-8. Abstract quote
To clarify the association of clinical and prognostic features with dermatomyositis (DM)-specific autoantibodies (Abs) in adult Japanese patients with DM.

Retrospective study.

Kanazawa University Graduate School of Medical Science Department of Dermatology and collaborating medical centers. Patients A total of 376 consecutive adult Japanese patients with DM who visited our hospital or collaborating medical centers between 2003 and 2008.

Clinical and laboratory characteristics of adult Japanese patients with DM and DM-specific Abs that include Abs against Mi-2, 155/140, and CADM-140.

In patients with DM, anti-Mi-2, anti-155/140, and anti-CADM-140 were detected in 9 (2%), 25 (7%), and 43 (11%), respectively. These DM-specific Abs were mutually exclusive and were detected in none of 34 patients with polymyositis, 326 with systemic sclerosis, and 97 with systemic lupus erythematosus. Anti-Mi-2 was associated with classical DM without interstitial lung disease or malignancy, whereas anti-155/140 was associated with malignancy. Patients with anti-CADM-140 frequently had clinically amyopathic DM and rapidly progressive interstitial lung disease. Cumulative survival rates were more favorable in patients with anti-Mi-2 compared with those with anti-155/140 or anti-CADM-140 (P < .01 for both comparisons). Nearly all deaths occurred within 1 year after diagnosis in patients with anti-CADM-140. Conclusion Dermatomyositis-specific Abs define clinically distinct subsets and are useful for predicting clinical outcomes in patients with DM.

A new approach to the classification of idiopathic inflammatory myopathy: myositis-specific autoantibodies define useful homogeneous patient groups

Love LA, Leff RL, Fraser DD, Targoff IN, Dalakas M, Plotz PH, Miller FW.

National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, Maryland 20892.

Medicine (Baltimore) 1991 Nov;70(6):360-74 Abstract quote

The IIM are a heterogeneous group of systemic rheumatic diseases which share the common features of chronic muscle weakness and mononuclear cell infiltrates in muscle. A number of classification schemes have been proposed for them, but none takes into consideration the marked immunologic, clinical, and genetic heterogeneity of the various clinical groups.

We compared the usefulness of myositis-specific autoantibodies (anti-aminoacyl-tRNA synthetases, anti-SRP, anti-Mi-2 and anti-MAS) to the standard clinical categories (polymyositis, dermatomyositis, overlap myositis, cancer-associated myositis, and inclusion body myositis) in predicting clinical signs and symptoms, HLA types, and prognosis in 212 adult IIM patients.

Although patients with inclusion body myositis (n = 26) differed in having significantly more asymmetric and distal weakness, falling, and atrophy than other patients, there were few other significant differences among the other clinical groups. In contrast, autoantibody status defined distinct sets of patients and each patient had only 1 myositis-specific autoantibody. Patients with anti-amino-acyl-tRNA synthetase autoantibodies (n = 47), compared to those without these antibodies, had significantly more frequent arthritis, fever, interstitial lung disease, and "mechanic's hands"; HLA-DRw52; higher mean prednisone dose at survey, higher proportion of patients receiving cytotoxic drugs, and higher death rates. Those with anti-signal recognition particle antibodies (n = 7) had increased palpitations; myalgias; DR5, DRw52; severe, refractory disease; and higher death rates. Patients with anti-Mi-2 antibodies (n = 10) had increased "V-sign" and "shawl-sign" rashes, and cuticular overgrowth; DR7 and DRw53; and a good response to therapy. The 2 patients with anti-MAS antibodies were the only ones with alcoholic rhabdomyolysis preceding myositis; both had insulin-dependent diabetes mellitus, and both had HLA-B60, -C3, -DR4, and -DRw53. These findings suggest that myositis-specific autoantibody status is a more useful guide than clinical group in assessing patients with myositis, and that specific associations of immunogenetics, immune responses, and clinical manifestations occur in IIM. Thus the myositis-specific autoantibodies aid in interpreting the diverse symptoms and signs of myositis patients and in predicting their clinical course and prognosis.

We propose, therefore, that an adjunct classification of the IIM, based on the myositis-specific autoantibody status, be incorporated into future studies of their epidemiology, etiology, and therapy.

Humoral immunity in polymyositis/

Targoff IN.

University of Oklahoma Health Sciences Center, Oklahoma City.

J Invest Dermatol 1993 Jan;100(1):116S-123S Abstract quote

Autoantibodies are found in most patients with polymyositis (PM) or dermatomyositis (DM) and 35-40% of these patients have myositis-specific antibodies. Twenty-five to thirty percent have anti-aminoacyl-tRNA synthetases, of which anti-Jo-1, directed at histidyl-tRNA synthetase, is by far the most common.

Patients with anti-synthetases have a high frequency of myositis, interstitial lung disease, Raynaud's phenomenon, and other features constituting an "anti-synthetase syndrome." Anti-synthetases tend to react with conformational epitopes and to inhibit enzymatic activity, suggesting reaction with conserved regions. Sera with antibodies to alanyl-tRNA synthetase (anti-PL-12) also have antibodies to tRNA(ala), whereas most sera with other anti-synthetases do not react directly with tRNA. Production of the antibodies appears to be antigen-driven, and is influenced by HLA genes, although an initiating factor, possibly a viral infection, may be important. Antibodies to other cytoplasmic antigens, most notably the signal recognition particle (anti-SRP), are seen in a small percentage of patients. Patients with anti-SRP do not tend to develop the anti-synthetase syndrome, but may have very severe disease. Antibodies to the nuclear antigen Mi-2 are also specific for myositis, and are strongly associated with DM. Several autoantibodies, including anti-PM-Scl, anti-Ku, and anti-U1 and U2 RNP, have been associated with scleroderma-PM overlap.

The role of humoral immunity in the myositis of PM and DM has not yet been clarified. Capillary loss and ischemic damage are important in DM, and seem to be mediated by humoral mechanisms, whereas cell-mediated attack on muscle fibers is important in PM. The mechanism of skin injury in cutaneous lesions is not known, but antibody deposition is inconsistent and uncommon. Whether the myositis-specific antibodies are involved in disease pathogenesis is not yet known, although there is no direct evidence for this. An understanding of the reasons for production of these antibodies, however, should provide insight into the etiology and pathogenesis of PM and DM.

Autoantibody profiles in the sera of European patients with myositis.

Brouwer R, Hengstman GJ, Vree Egberts W, Ehrfeld H, Bozic B, Ghirardello A, Grondal G, Hietarinta M, Isenberg D, Kalden JR, Lundberg I, Moutsopoulos H, Roux-Lombard P, Vencovsky J, Wikman A, Seelig HP, van Engelen BG, van Venrooij WJ.

Department of Biochemistry, University of Nijmegen, Nijmegen, The Netherlands.

Ann Rheum Dis 2001 Feb;60(2):116-23 Abstract quote

OBJECTIVE: To determine the prevalence of myositis specific autoantibodies (MSAs) and several myositis associated autoantibodies (MAAs) in a large group of patients with myositis.

METHODS: A total of 417 patients with myositis from 11 European countries (198 patients with polymyositis (PM), 181 with dermatomyositis (DM), and 38 with inclusion body myositis (IBM)) were serologically analysed by immunoblot, enzyme linked immunosorbent assay (ELISA) and/or immunoprecipitation.

RESULTS: Autoantibodies were found in 232 sera (56%), including 157 samples (38%) which contained MSAs. The most commonly detected MSA was anti-Jo-1 (18%). Other anti-synthetase, anti-Mi-2, and anti-SRP autoantibodies were found in 3%, 14%, and 5% of the sera, respectively. A relatively high number of anti-Mi-2 positive PM sera were found (9% of PM sera). The most commonly detected MAA was anti-Ro52 (25%). Anti-PM/Scl-100, anti-PM/Scl-75, anti-Mas, anti-Ro60, anti-La, and anti-U1 snRNP autoantibodies were present in 6%, 3%, 2%, 4%, 5%, and 6% of the sera, respectively. Remarkable associations were noticed between anti-Ro52 and anti-Jo-1 autoantibodies and, in a few sera, also between anti-Jo-1 and anti-SRP or anti-Mi-2 autoantibodies.

CONCLUSIONS: The incidence of most of the tested autoantibody activities in this large group of European patients is in agreement with similar studies of Japanese and American patients. The relatively high number of PM sera with anti-Mi-2 reactivity may be explained by the use of multiple recombinant fragments spanning the complete antigen. Furthermore, our data show that some sera may contain more than one type of MSA and confirm the strong association of anti-Ro52 with anti-Jo-1 reactivity.

Prevalence of 52-kd and 60-kd Ro/SS-A autoantibodies in Japanese patients with polymyositis/

Kubo M, Ihn H, Asano Y, Yamane K, Yazawa N, Tamaki K.

Department of Dermatology, Faculty of Medicine, University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan.

J Am Acad Dermatol 2002 Jul;47(1):148-51 Abstract quote

To determine the prevalence of 52-kd and 60-kd Ro/SS-A antibodies in Japanese patients with polymyositis/dermatomyositis, we examined serum samples from 61 patients with PM/DM, 10 patients with primary Sjogren's syndrome, and 25 healthy control subjects. Six serum samples possessed anti-Ro/SS-A antibodies and were positive for anti-Ro52, anti-Ro60, or both. Two reacted with both Ro52 and Ro60, and 4 reacted with Ro52 alone.

The results suggest that Ro52 is the main antigen of anti-Ro/SS-A antibodies in patients with polymyositis/dermatomyositis and that its coexistence with other defined antibodies suggests the existence of a subgroup of patients with various serologic abnormalities.


GENERAL Curr Opin Rheumatol 1999;11:475-482

Classic DM
Classic DM with malignancy
Classic DM as part of an overlapping connective tissue disorder

Clinically amyopathic DM
Clinically hypomyopathic DM

Juvenile-onset Classic DM
Clinically amyopathic DM
Clinically hypomyopathic DM
Polymyositis Isolated polymyositis
Polymyositis as part of an overlapping connective tissue disorder
Polymyositis associated with internal malignancy (?)
Inclusion body myositis  
A systematic review of adult-onset clinically amyopathic dermatomyositis (dermatomyositis sine myositis): a missing link within the spectrum of the idiopathic inflammatory myopathies.

Gerami P, Schope JM, McDonald L, Walling HW, Sontheimer RD.

Dermatopathology Division, University of Chicago, Chicago, Illinois, USA.

J Am Acad Dermatol. 2006 Apr;54(4):597-613. Epub 2006 Jan 23. Abstract quote  

OBJECTIVE: Classical dermatomyositis (CDM) patients display the hallmark cutaneous manifestations of dermatomyositis (DM), proximal muscle weakness, and laboratory evidence of myositis. The epidemiology and management of both adult-onset and juvenile-onset CDM has been well characterized. However, the clinical significance of the hallmark inflammatory cutaneous manifestations of DM occurring in individuals who have no clinically significant muscle weakness and normal muscle enzymes for prolonged periods of time (ie, 6 months or longer) has not been clear. The term amyopathic DM (ADM) (synonymous with DM sine myositis) has been proposed to draw attention to such individuals. A related form of DM, "hypomyopathic DM" [HDM], is the presence of DM skin disease for 6 months or longer in individuals who have no muscle weakness but who are found to have some evidence of muscle inflammation upon testing (muscle enzyme levels, electromyogram, muscle biopsy, muscle magnetic resonance imaging [MRI]). Clinically amyopathic DM (CADM) is a designation that has been proposed for patients having either ADM or HDM. The clinically amyopathic component of this designation was coined to emphasize the fact that the only clinical problem being experienced by these patients at the time of diagnosis is their DM skin disease. Our personal experience suggests that the CADM subphenotype might be more prevalent in adults than has been thought previously. To test this hypothesis and address questions relating to the optimal management and prognosis of such patients, we have systematically reviewed the published literature in this area.

METHODS: We carried out a systematic review of the published literature on adult-onset CADM as defined in Table 1 through May 1, 2004.

RESULTS: We identified 291 adult-onset CADM cases (18 years or older) reported from over 19 countries. The average duration of DM skin disease was 3.74 years (range, 6 months [by definition] to > 20 years), and 73% were female. Among 37 patients with HDM who were identified, the average duration of disease was 5.4 years, and none had developed clinically significant weakness at the time of the reports. Thirty-seven of the reported CADM patients developed muscle weakness greater than 6 months after onset of their skin disease (15 months to 6 years). For the sake of this discussion, such patients have been analyzed under the designation of "CADM --> CDM." Somewhat surprisingly, 36/291 (13%) of the identified published CADM patients developed interstitial lung disease. Incidental to our review, we also identified 10 published cases of individuals having DM skin disease and interstitial lung disease without muscle weakness, 7 of whom died from interstitial lung disease less than 6 months after onset of their DM skin disease (the term pre-myopathic DM coined by others has been used here to refer to such patients). In addition, an associated internal malignancy was found in 41/291 (14%) of the identified CADM cases. A positive antinuclear antibody was reported in 63% and myositis-specific autoantibodies (eg, Jo-1, Mi-2) in only 3.5% of the reported CADM patients in which such data were available.

CONCLUSIONS: The results of this analysis suggests that the CADM subphenotype is more common than has been thought previously and that such patients may comprise a relatively high proportion of DM patients followed by dermatologists. Some CADM patients also have been observed to develop overt proximal muscle weakness years after onset of their DM skin disease. In addition, CADM patients appear to be at risk of developing the same potentially fatal disease associations/complications for which CDM patients are at risk (eg, interstitial lung disease and internal malignancy). Population-based studies of the epidemiology and optimal management of CADM patients, including efforts to identify risk factors associated with potentially fatal outcomes such as late-onset muscle weakness, interstitial lung disease, and malignancy, are needed. As an incidental finding to this literature review, we also identified a small number of reported cases of often-fatal interstitial lung disease occurring shortly after the onset of DM skin disease (< 6 months) in the complete absence of muscle weakness. This subphenotype, referred to as "pre-myopathic DM," is one with which dermatologists should be aware as early diagnosis and aggressive management can be lifesaving.

Amyopathic dermatomyositis (dermatomyositis sine myositis). Presentation of six new cases and review of the literature.

Euwer RL, Sontheimer RD.

Department of Dermatology, University of Texas Southwestern Medical Center, Dallas, TX 75235.

J Am Acad Dermatol 1991 Jun;24(6 Pt 1):959-66 Abstract quote

We report six patients with the classic cutaneous findings of dermatomyositis who did not develop clinical or laboratory evidence of muscle disease for at least 2 years after onset of their skin manifestations.

Such patients represent 11% of our total experience with dermatomyositis patients during a 15 year period. All six patients had Gottron's paules, periungual erythema/telangiectasia, and violaceous discoloration of the face, neck, upper chest, and back at some time during the course of their disease. In addition, all complained of pruritus and photosensitivity. None of the patients had evidence of malignancy. Each of five patients treated with oral corticosteroids for their cutaneous disease had marked improvement and did not develop myositis.

These cases further emphasize that the cutaneous manifestations of dermatomyositis are pathognomonic of this disease and challenge the commonly held notion that muscle disease always develops within 2 years of onset of skin disease.

Juvenile amyopathic dermatomyositis.

Schmid MH, Trueb RM.

Department of Dermatology, University Hospital of Zurich, Switzerland.

Br J Dermatol 1997 Mar;136(3):431-3 Abstract quote

We report a 15-year-old girl with a 10-year-old history of typical skin features of dermatomyositis (DM) without evidence of muscle involvement.

Amyopathic dermatomyositis (ADM) is defined by the presence of biopsy confirmed classic cutaneous findings of dermatomyositis in the absence of any clinical or laboratory signs of muscle disease for at least 2 years after onset of skin pathology. To exclude muscle involvement muscle enzymes should be normal; moreover additional use of magnetic resonance imaging and muscle ultrasound is currently being proposed. It is as yet undetermined, whether early aggressive immunosuppressive treatment of ADM might prevent the development of myositis at a later date or influence the course of the skin disease.

In a paediatric patient with ADM we advocate a more expectant attitude with careful and regular monitoring for possible development of muscle disease.

Juvenile amyopathic dermatomyositis: results of a case finding descriptive survey.

Plamondon S, Dent PB.

Department of Pediatrics, McMaster University, Children's Hospital, Hamilton Health Sciences Corporation, Ontario, Canada.

J Rheumatol 2000 Aug;27(8):2031-4 Abstract quote

OBJECTIVE: To review the clinical features of juvenile amyopathic dermatomyositis (ADM) to define an appropriate approach to its diagnosis and management.

METHODS: Based on a review of published adult and pediatric cases, a prevalidated, peer reviewed, 3 page questionnaire was sent to all members of the Pediatric Section of the American College of Rheumatology and American Society for Pediatric Dermatology.

RESULTS: Thirty-nine questionnaires were submitted for analysis. Twelve cases were excluded due to abnormal test results. Only one case met all criteria. Although 26 cases were incompletely investigated or had inadequate followup, they were not excluded, as all completed tests were normal. Two patients with incomplete data developed calcinosis. Of 27 patients not positively excluded, 10 were treated systemically, with 5 achieving remission, while 11/17 untreated recovered spontaneously. At a mean followup of 32.8 months from disease onset none of the 27 patients has developed clinical myopathy.

CONCLUSION: The classic skin changes of juvenile DM can occur in the absence of clinical muscle involvement. Physicians are not routinely performing electromyography, muscle biopsy, or magnetic resonance imaging in the assessment of these patients. A significant proportion of patients with ADM will remit without systemic therapy. Optimum treatment needs to be determined through controlled trials.

Amyopathic dermatomyositis.

Olsen NJ, Park JH, King LE.

Division of Rheumatology, Department of Medicine, Vanderbilt University, T-3219 Medical Center North, Nashville, TN 37232, USA.

Curr Rheumatol Rep 2001 Aug;3(4):346-51 Abstract quote

Amyopathic dermatomyositis is a variant of dermatomyositis that is characterized by the typical skin rash but without the muscle abnormalities.

It has been proposed that the amyopathic and myopathic forms of dermatomyositis exist on a continuum, a concept that is supported by family and genetic studies and the observation that a small proportion of amyopathic patients transform to a frankly myopathic state. The amyopathic state is defined by a lack of muscle weakness and through diagnostic tests, including serum muscle enzymes, electromyogram studies, and muscle biopsies, that are usually normal or show only minimal abnormalities. Despite the lack of weakness, many patients complain of debilitating fatigue. More sensitive measures of muscle function, such as P-31 magnetic resonance spectroscopy, suggest that muscle metabolism is abnormal in amyopathic patients. The amyopathic form is more commonly seen in adults than in children, although juvenile cases are reported. Some early series suggested no association with underlying malignancies, but recent reports indicate that malignancies occur.

Determining whether a patient has amyopathic rather than myopathic disease may have prognostic implications.

Amyopathic Dermatomyositis A Review by the Italian Group of Immunodermatology

Marzia Caproni, MD; Carla Cardinali, MD; Aurora Parodi, MD; Barbara Giomi, MD; Manuela Papini, MD; Mario Vaccaro, MD; Angelo Marzano, MD; Clara De Simone, MD; Marcello Fazio, MD; Alfredo Rebora, MD; Paolo Fabbri, MD

Arch Dermatol. 2002;138:23-27 Abstract quote

Objective To analyze the average age, sex distribution, duration of follow-up, clinical course, serologic abnormalities, and incidence of possibly associated malignancy in patients with amyopathic dermatomyositis.

Design Retrospective study.

Setting University hospitals.

Patients Thirteen patients with amyopathic dermatomyositis.

Results The 13 patients represented 8.2% of 157 patients with dermatomyositis seen retrospectively in a 10-year period by the Italian Group of Immunodermatology of the Italian Society of Dermatology and Venereology. Gottron papules and sign and periungual telangiectasias were found in approximately 50% of cases (papules in 7 patients, Gottron sign and periungual telangiectasias in 6), while periorbital violaceous erythema was seen in 70% (9 patients). Arthralgias occurred in 2 patients and Raynaud phenomenon in 4. An elevated erythrocyte sedimentation rate was detected in 6 patients, hepatitis B virus antigen in 3, speckled antinuclear antibodies in 7, and anti-Ro and antimitochondrial antibodies in 1 case each. None of our patients had evidence of internal malignancy. Neither cardiopulmonary nor esophageal dysfunction was demonstrated. Electromyography showed a protopathic muscle abnormality in 3 patients. Muscle biopsy disclosed myositis and a neurogenic myopathy in another one.

Conclusions Amyopathic dermatomyositis is a rare disease. So far, only 2 series of a few cases each have been reported. The "amyopathic" subset of dermatomyositis is peculiar in that its cutaneous lesions are predominant for long periods or even permanently, although they are indistinguishable from those of classic dermatomyositis. The minimal or absent muscle disease and the rarity of serum immunologic findings imply a favorable prognosis in white patients.

Amyopathic dermatomyositis: Retrospective review of 37 cases.

el-Azhary RA, Pakzad SY.

Department of Dermatology, Mayo Clinic, Rochester.

J Am Acad Dermatol 2002 Apr;46(4):560-5 Abstract quote

Criteria for diagnosis of amyopathic dermatomyositis vary, and the prognosis is not clear. Our purpose was to investigate prognosis regarding progression to myositis and associated malignancy.

We reviewed the medical records of patients with dermatomyositis evaluated at our institution from 1976 to 1994. Of 746 patients with dermatomyositis, 37 (5%) with the amyopathic subtype were divided into 3 groups: group 1 (73%), no subjective or objective evidence of myopathy; group 2 (13%), no subjective muscle weakness but abnormalities detected by objective tests; group 3 (13%), subjective muscle weakness but no objective evidence of myopathy. Follow-up was conducted by means of a mailed questionnaire. For 25 patients, follow-up of 1 to 17 years after diagnosis showed muscle weakness in 2 patients in group 1 within 5 years after diagnosis. Five patients (13%) had associated malignancies. Of 7 (19%) patients with disease onset before the age of 18 years, none had progression to myopathy.

Although it presents with cutaneous lesions indistinguishable from those of classic dermatomyositis, amyopathic dermatomyositis is a distinct entity. In most patients, amyopathic dermatomyositis does not progress to myopathy. Prognosis appears favorable, but malignancy may develop.


Inclusion body myositis: clinical and histopathological features of 36 patients.

Beyenburg S, Zierz S, Jerusalem F.

Neurologische Universitatsklinik Bonn.

Clin Investig 1993 May;71(5):351-61 Abstract quote

Thirty-six patients (15 females, 21 males) with inclusion body myositis (IBM) were studied.

The diagnosis was established according to clinical and histopathological criteria. Clinical features were insidious onset of slowly progressive muscle weakness and wasting with depressed or absent tendon reflexes especially in the lower limbs. The pattern of muscle weakness was variable. The majority of patients (58%) showed proximal and symmetrical weakness usually most prominent in the legs. Isolated distal (6%) and asymmetrical weakness (19%) was less frequently observed. Myalgia occurred in 42% of the patients. The age at onset of symptoms ranged from 20 to 73 years (mean 47 years). Serum creatine kinase levels were normal (11%) or mildly elevated (89%). Needle electromyography revealed myopathic features in about 80% of the patients, and results of nerve conduction studies were normal in most of the cases. The predominant histopathological findings were numerous muscle fibers with rimmed vacuoles (100% of the patients), groups of atrophic fibers (92%), and inflammatory infiltrates (89%). The inflammatory infiltrates were located predominantly at endomysial sites and were composed mainly of T8 cells. Electron microscopy showed characteristic intracytoplasmic filamentous inclusions in all 36 cases. Immunosuppressive treatment in 16 patients failed to prevent disease progression in all but one patient with an associated Sjogren's syndrome.

It is concluded that the consistent combination of typical histopathological findings and characteristic clinical features offers a firm basis for the diagnosis of IBM. IBM should be suspected in any adult patient presenting with clinical signs of a chronic polymyositis unresponsive to immunosuppressive therapy. The etiology and pathogenesis of IBM remain to be established.

JUVENILE Usually greater incidence of multiorgan disease with rarely a necrotizing vasculitis
Calcification within skeletal muscle common

Juvenile dermatomyositis: a retrospective review of a 30-year experience.

Peloro TM, Miller OF 3rd, Hahn TF, Newman ED.

Department of Dermatology, Geisinger Health System, Danville, Pennsylvania, USA.

J Am Acad Dermatol 2001 Jul;45(1):28-34 Abstract quote

OBJECTIVE: Our purpose was to evaluate the epidemiology trends, presenting clinical features, laboratory data, and outcome of patients with JDMS.

METHODS: A total of 16 patients were identified at Geisinger Medical Center by a 30-year retrospective chart review.

RESULTS: Sex ratio, age at diagnosis, and outcome were similar to data published in previous studies. However, certain trends were noted. The most common initial physical examination findings were an extremity rash (94%) and periungual erythema (75%). New associations of JDMS that were uncovered included the findings of pruritus (38%) and a psoriasiform scalp dermatitis (25%). Nonspecific laboratory elevations were the most common initial laboratory changes (erythrocyte sedimentation rate, lactate dehydrogenase, and aspartate aminotransferase). Tubuloreticular inclusions as found on electron microscopy of muscle biopsy specimens were present in all 3 patients tested. One patient with tubuloreticular inclusions had otherwise normal muscle biopsy findings on hematoxylin-and-eosin staining. Two of the 16 patients had cutaneous findings of JDMS but did not exhibit muscle involvement after long-term follow-up at 4 and 5 years.

CONCLUSION: Our study confirms that the initial physical and laboratory findings in patients with JDMS may be nonspecific. The heliotrope rash and Gottron papules classically associated with dermatomyositis appeared less commonly than an extremity rash and periungual erythema. Creatinine kinase and aldolase levels may not be elevated on initial presentation. Pruritus, a psoriasiform scalp dermatitis, and tubuloreticular inclusions found on muscle biopsy electron microscopy should be additional factors to consider. The long-term follow-up in 2 patients without muscle involvement lends support to the existence of amyopathic dermatomyositis.

Lipodystrophy in patients with juvenile dermatomyositis--evaluation of clinical and metabolic abnormalities.

Huemer C, Kitson H, Malleson PN, Sanderson S, Huemer M, Cabral DA, Chanoine JP, Petty RE.

Department of Pediatrics, University of British Columbia, Vancouver, Canada.

J Rheumatol 2001 Mar;28(3):610-5 Abstract quote

OBJECTIVE: Lipodystrophy and associated metabolic abnormalities are being increasingly recognized as complications of juvenile dermatomyositis (JDM). We investigated the prevalence of lipodystrophy and the extent of metabolic abnormalities related to lipoatrophic diabetes mellitus in patients with JDM.

METHODS: Twenty patients with JDM were evaluated for evidence of lipodystrophy and associated lipoatrophic diabetes mellitus. All patients underwent clinical assessment, laboratory investigations, and metabolic studies (oral glucose tolerance test, lipid studies, insulin antibodies).

RESULTS: We found clinical evidence of lipodystrophy and lipoatrophic diabetes mellitus in 4 of 20 patients with JDM and metabolic abnormalities known to be associated with lipodystrophy in another 8 patients. The 20 patients with JDM were categorized as follows: Group 1 (Patients 1-4) consisted of patients with lipodystrophy and either diabetes mellitus (2 patients) or impaired glucose tolerance (2 patients); Group 2 (Patients 5-12): no lipodystrophy but abnormal glucose and/or lipid studies; Group 3 (Patients 13-20): no lipodystrophy and no abnormalities of glucose and lipid studies.

CONCLUSION: We found 25% of patients with JDM have lipodystrophy, and 50% present with hypertriglyceridemia and insulin resistance. Screening for metabolic abnormalities in JDM should be included in routine followup because of the effect of lipodystrophy on longterm prognosis.


Dermatomyositis with a pityriasis rubra pilaris-like eruption: a little-known distinctive cutaneous manifestation of dermatomyositis.

Requena L, Grilli R, Soriano L, Escalonilla P, Farina C, Martin L.

Department of Dermatology, Fundacion Jimenez Diaz, Universidad Autonoma, Madrid, Spain.

Br J Dermatol 1997 May;136(5):768-71 Abstract quote

A pityriasis rubra pilaris-like eruption has been described in patients with dermatomyositis. These patients showed generalized follicular hyperkeratosis and diffuse thickening of the palms and soles. Histopathological findings consisted of keratotic plugging of the follicular infundibulum and features of erector pili myositis.

We report on an 18-year-old woman with dermatomyositis. The diagnosis was established by characteristic enzymatic alterations, electromyographic pattern of myositis and the findings in a muscle biopsy, although the patient had no evidence of muscular weakness during a follow-up of 14 years. She developed an erythematosus and squamous eruption associated with diffuse palmoplantar keratoderma.

Histopathological features consisted of a papillomatous epidermis with spicules of compact eosinophilic hyperkeratosis over the tips of papillae that were not related to hair follicles. Pityriasis rubra pilaris-like eruption seems to be a characteristic although uncommon cutaneous manifestation in dermatomyositis.

An unusual presentation of dermatomyositis: the type Wong variant revisited.

Lupton JR, Figueroa P, Berberian BJ, Sulica VI.

The George Washington University Medical Center, 2311 M St. NW, Washington, DC 20037, USA.

J Am Acad Dermatol 2000 Nov;43(5 Pt 2):908-12 Abstract quote

We describe a 53-year-old white woman with dermatomyositis (DM) who had additional clinical findings of pityriasis rubra pilaris (type Wong dermatomyositis) with histopathologic features of both pityriasis rubra pilaris (PRP) and porokeratosis.

Type Wong dermatomyositis was originally described in 11 patients by Wong in 1969 and has been reported in 5 additional patients. This is a rarely described phenomenon in which patients with DM develop cutaneous hyperkeratotic lesions that resemble PRP and histologically show follicular hyperkeratosis and hair follicle destruction. Arrector pilorum muscles also show degenerative findings and myositis.

We believe that this is the first reported case of a patient with type Wong DM who also has clinical and histologic features suggestive of porokeratosis. This is important because of the association of adult-onset dermatomyositis with internal malignancy and the well-documented association of porokeratosis with immunosuppression. These clinical and histologic findings serve as markers for malignancy in patients with DM. These patients warrant a complete review of systems and investigation for age-appropriate neoplasms as well as close long-term follow-up by dermatologists to ensure that these cutaneous eruptions are not overlooked.


Dermatomyositis: a clinicopathological study of 40 patients.

Department of Dermatology, Wake Forest University Baptist Medical Center, Winston-Salem, NC 27157, USA.


Am J Dermatopathol. 2009 Feb;31(1):61-7. Abstract quote

The histopathology of cutaneous lesions of dermatomyositis (DM) may be indistinguishable from acute cutaneous lesions of systemic lupus erythematosus (SLE). Misreported or incomplete clinical information may result in a clinicopathologic discrepancy and a delay in making a correct diagnosis of DM.

The aim of this study was to systematically characterize the histopathologic findings of cutaneous lesions of DM and to determine if skin biopsy specimens of DM and SLE could be distinguished by light microscopic examination. Biopsies from 40 patients diagnosed with DM at the Wake Forest University School of Medicine from 1994 to 1999 were reviewed. The histological features by light microscopy were graded in a systematic fashion. We then assessed whether the cutaneous pathological changes of DM could be distinguished from those of SLE. Ten biopsy specimens each of DM and SLE (matched for anatomical site and lesion morphology) were randomized.

Histological grading was performed in a blinded fashion, as was a histopathologic diagnosis (DM versus SLE). The most consistent histological findings of DM included increased dermal mucin, vacuolar alteration of the basal cell layer, and mild-to-moderate mononuclear cell inflammatory infiltrates.

Our results show that the histological grading of SLE skin biopsies was nearly identical to that of DM. The correct histopathologic diagnosis of DM or SLE was made in 11 of the 20 skin biopsies without clinical information.

Despite the limitations of our small sample size, these findings suggest that acute cutaneous lesions of SLE cannot be distinguished from DM. Clinicopathologic correlation is important for making a diagnosis of DM or SLE.
Cutaneous lesions of dermatomyositis with supervening fibrosis.

Department of Pathology, New York Presbyterian Hospital/Weill Medical College of Cornell University, New York, NY, USA.


J Cutan Pathol. 2008 Jan;35(1):31-9. Abstract quote

Background: Dermatomyositis (DM) is a distinctive systemic connective tissue disease whereby the skin defines a cardinal site of involvement. There exists a body of literature, which suggests that a significant component of its clinical manifestations may be related to endothelial cell injury. We have postulated in the past that anti-endothelial cell antibodies may be the defining trigger leading to endothelial cell dysfunction. The primary organs affected by DM are the skin and muscle. A significant albeit rare complication is pulmonary fibrosis, which our recent study postulated to be attributable to an autoimmune endothelialitis.

Design: We describe six patients, four women and two men who ranged in age from 3 to 60 years, and had classic clinical presentations and cutaneous lesions of DM without any supervening clinical changes indicative of cutaneous sclerosis.

Results: Skin biopsies showed cell-poor interface dermatitis with variable dermal mucin and C5b-9 within the cutaneous vasculature. However, at variance with classic DM was the presence of a sclerodermoid tissue reaction, which was of variable depth. All of these patients had severe muscle involvement. One pediatric patient had concomitant significant cutaneous, central nervous system and oral mucosal ischemic infarcts. Significant pulmonary disease ensued in the four adult patients, manifesting as pulmonary fibrosis in two, diffuse alveolar damage in one and diaphragmatic failure in one. In three patients, direct immunofluorescent studies were corroborative of immune-based microvascular injury while Western blot and/or indirect immunofluorescent studies showed anti-endothelial cell antibody activity within the serum of three patients.

Conclusions: The identification of sclerosis in biopsies of skin lesions typical clinically for DM may be a harbinger for more severe autoimmune-based endothelial cell injury phenomenon. One could speculate that its basis may be attributable to elevated serum levels of the natural fibrogenic factor, transforming growth factor beta, which in turn is released from damaged endothelium.
Histopathology of Gottron's papules - utility in diagnosing dermatomyositis.

UMASS Medical School, 55 Lake Avenue North, Worcester, MA, USA.


J Cutan Pathol. 2007 Oct;34(10):793-6. Abstract quote

Dermatomyositis (DM) is an uncommon connective tissue disease that presents with a characteristic violaceous skin eruption as well as proximal muscle weakness, primarily of the upper extremities. Cutaneous stigmata of DM include Gottron's papules, similarly colored papules and plaques overlying the extensor surfaces of finger joints. While biopsy of the typical poikilodermatous skin eruption found in patients with suspected DM is a standard algorithmic component in the workup and diagnosis of the disease, Gottron's papules are rarely sampled for histopathologic assessment. The precise reason for this is not known but may be related to problems associated with healing because of constant motion forces in the vicinity of the joint. Given this, sparse literature is available on the histopathologic features of Gottron's papules.

In this study, we present two cases in which the presence of papular (Gottron's papules) lesions on the fingers led to a presumptive diagnosis of DM and prompted biopsies of the same. The study illustrates the diagnostic utility of biopsies from Gottron's papules.

Dermatomyositis and mucinosis.

del Pozo J, Almagro M, Martinez W, Yebra-Pimentel MT, Garcia-Silva J, Pena-Penabad C, Fonseca E.

Departments of Dermatology and Pathology, Hospital Juan Canalejo, La Coruna, Spain.

Int J Dermatol 2001 Feb;40(2):120-4 Abstract quote

BACKGROUND: Mucin deposition is a common feature in autoimmune collagen diseases including dermatomyositis. Nevertheless, clinical manifestations of mucinosis are uncommon in patients with dermatomyositis. Two cases of mucinosis associated with dermatomyositis are reported.

PATIENTS: A 53-year-old woman presented with symmetrical plaques on the upper limbs formed by the coalescence of small, violaceous papules. In addition, she showed the typical cutaneous and muscle features of dermatomyositis. A 44-year-old woman with dermatomyositis of 5 years' evolution developed linear, flesh-colored papules across the flexural creases of her palms and fingers.

RESULTS: Skin biopsy of the upper limb lesions in the first patient showed epidermal changes compatible with dermatomyositis and dermal mucin deposition. Histopathologic examination of the palmar lesions of the second patient showed less intense epidermal changes of dermatomyositis and dermal mucin deposition.

CONCLUSIONS: Mucin deposition in patients with dermatomyositis may have an unusual clinical presentation, and it should be considered in the differential diagnosis of atypical cutaneous lesions in these patients.


Cutaneous vasculitis in a patient with dermatomyositis without muscle involvement.

Kadoya A, Akahoshi T, Sekiyama N, Hosaka S, Kondo H.

Department of Internal Medicine, Kitasato University, School of Medicine, Sagamihara.

Intern Med 1994 Dec;33(12):809-12 Abstract quote

A 74-year-old female patient with cutaneous ulcerations and typical dermatomyositis (DM) skin rash had no muscle disease for a 1-year and 5 months period.

Histological examination of the skin ulceration indicated vascular occlusion without cellular infiltration.

Cutaneous ulceration is a very rare manifestation of adult-onset DM patients without inflammatory myopathy.

Cutaneous leukocytoclastic vasculitis in dermatomyositis suggests malignancy.

Hunger RE, Durr C, Brand CU.

Dermatological Clinic, Inselspital, University of Berne, Switzerland.

Dermatology 2001;202(2):123-6 Abstract quote

BACKGROUND: Dermatomyositis (DM) is a rare connective tissue disease which has been shown to be associated with an underlying malignancy.

OBJECTIVE: Evaluation of the prevalence of malignancy in DM at our clinic and search for characteristics of the paraneoplastic form of disease.

METHODS: Retrospective review of patient files and histology reports over the period from 1991 to 1998.

RESULTS: 23 patients (14 women and 9 men) with DM could be identified in this time period with a median age at diagnosis of 48 years. Malignancies were found in 5 (22%) cases. The skin biopsies of all patients showed features of DM; in 7 cases, a leukocytoclastic vasculitis was detected. Four of the 5 cases with an associated malignancy demonstrated histologically a vasculitis in lesional skin, compared to only 3 out of 18 cases without malignancy (p < 0.05, Fisher's exact test).

CONCLUSION: Our data suggest that vasculitis in lesional skin biopsies has a predictive value for the presence of underlying malignancy.


Quantitative histopathology of the inflammatory myopathies.

Ringel SP, Carry MR, Aguilera AJ, Starcevich JM.

Arch Neurol 1986 Oct;43(10):1004-9 Abstract quote

This study quantitatively assessed skeletal muscle histopathology in 57 patients with inflammatory myopathy, including 20 patients with polymyositis (PM), 19 patients with dermatomyositis (DM), and 18 patients with evidence of an additional connective-tissue disease. No histologic criteria for invariably distinguishing patients with inflammatory myopathy were established because of overlap in individual measurements, but general histopathologic distinctions were confirmed. In PM, endomysial mononuclear cell infiltration (fibers bordering on inflammation) was usual, whereas in DM inflammation of large vessels, fibers with circumscribed areas of myofibrillar loss, and perifascicular atrophy were seen. Patients with evidence of an additional connective-tissue disease were most similar to the DM patients, with a greater prevalence of perivascular inflammation than in the PM patients.

Because of varying histopathology (and presumed varying pathogenesis), future therapeutic trials would be more informative if they were designed using patients with homogeneous histologic features.



The immunofluorescent profile of dermatomyositis: a comparative study with lupus erythematosus.

Magro CM, Crowson AN.

Department of Pathology, Beth Israel Deaconess Medical Centre, Harvard Medical School, Cambridge, MA, USA.

J Cutan Pathol 1997 Oct;24(9):543-52 Abstract quote

We have demonstrated a role for microvascular injury mediated by the membrane attack complex of complement (C5b-9) in the genesis of cutaneous lesions of dermatomyositis (DM) (1).

The purpose of this study is to revisit the immunofluorescent (IF) profile of DM, to further investigate the role of C5b-9 in the pathogenesis of cutaneous lesions, and to see if any features of the IF profile reliably distinguish DM from LE. Lesional skin biopsies from 24 patients with clinical findings characteristic of DM were received in formalin and in Michel's transport medium. Conventional light microscopy, and IF studies with antibodies monospecific for IgG, IgA, IgM, C3, fibrin and C5b-9 were performed. The control group comprised biopsies from 31 patients with well-documented LE.

A positive lupus band test (LBT) correlated highly with a diagnosis of LE, with a sensitivity of 64.5% and a specificity of 95.6% (p=0.001). The LBT was most sensitive in the setting of DLE and SLE and was least sensitive in the setting of SCLE.

The finding of vascular C5b-9 deposition correlated with a diagnosis of DM versus LE (p=0.001) although the false positive rate was 21.4%. The false negative rate was reduced when vascular C5b-9 was seen in the absence of antibodies to Ro, La, or RNP. While a negative LBT correlated with a diagnosis of DM (p=0.001), the specificity was only 64.5%. However, when it was seen in concert with C5b-9 along the DEJ, specificity was increased to 80.6% (p=0.001).

The presence of C5b-9 in vessels and along the DEJ in concert with a negative LBT was predictive of DM (p=0.001) with a specificity of 93.5%, sensitivity of 78.3%, a false positive rate of 10% and a false negative rate of 14.7%.

The combination of a negative LBT, vascular C5b-9 deposition and negative serology for Ro, La, and RNP was a predictor of DM versus LE with a sensitivity of 90.5%, a specificity of 96.8%, a false positive rate of 5% and a false negative rate of 6.2% (p=0.001).

The IF profile of DM in lesional skin comprises a negative LBT, deposition of C5b-9 within vessels and along the DEJ, and variable keratinocyte decoration for IgG and C5b-9. The most statistically powerful predictor of DM is the combination of a negative LBT with vascular C5b-9 deposition and negative serology for antibodies to Ro, La, Sm, and RNP. Demonstration of a negative LBT in all but 1 case of DM suggests that the DEJ is not a primary site for antigen-antibody interaction.

We postulate that the aforementioned IF findings reflect humorally mediated injury of endothelium and keratinocytes, effected by C5b-9.



Acute inflammatory myopathy with severe subcutaneous edema, a new variant? Report of two cases and review of the literature.

Gorelik O, Almoznino-Sarafian D, Alon I, Rapoport MJ, Goltsman G, Herbert M, Modai D, Cohen N.

Department of Internal Medicine F, Assaf Harofeh Medical Center, Tel-Aviv University, Zerifin, Israel.

Rheumatol Int 2001 May;20(4):163-6 Abstract quote

Acute inflammatory myopathy with severe subcutaneous edema is extremely rare and has been reported in only a handful of cases.

We describe two similar patients presenting with this disorder and generalized rash.

Unlike the five previously reported cases, the clinical and histologic features of our two patients are more suggestive of dermatomyositis than polymyositis. Nevertheless, scrutinizing all seven reported patients, a number of specific characteristics could be defined. All patients were adult males. Dysphagia was present in four. In six patients, acute inflammatory myopathy was idiopathic while malignancy was present in one. Two patients died despite intensive therapy, three improved on corticosteroid treatment, and two recovered spontaneously. In all patients, limb involvement with marked subcutaneous edema was present, clinically mimicking deep vein thrombosis in both our patients.

The presence of severe subcutaneous edema may be a hallmark of a distinctive variant of acute inflammatory myopathy. More cases are needed to discern subtypes of this general entity and to establish guidelines for treatment and prognosis.


Skin metastasis of breast cancer clinically undistinguished from amyopathic dermatomyositis.

Seishima M, Shimizu H, Oyama Z.

Department of Dermatology, Ogaki Municipal Hospital, Minaminokawa-cho 4-86, Ogaki, 503-8502, Japan.

Eur J Dermatol 2001 Mar-Apr;11(2):131-3 Abstract quote

We report a 65-year-old woman who consulted us on May 25, 1998, showing pruritic, partially flagellate erythema on the back and upper extremities, livedo lesions with erythema on the loins, and erythematous papules on the dorsal finger joints for 2 months.

Histopathological findings of erythema on the back showed mononuclear cell infiltration around capillaries and marked edema in the dermis. Laboratory data were within normal range except for positive anti-nuclear antibody. She had undergone total left mastectomy on June 2, 1997 for breast cancer. Supraclavicular lymph node metastasis was found at the beginning of May, 1998. A diagnosis of amyopathic dermatomyositis associated with breast cancer was made. Erythema with itching gradually subsided from the end of August, 1998. Treatment with radiation and chemotherapy reduced lymph node swelling, but complete remission was not obtained. Erythema similar to the previous lesion but without itching re-appeared on the back from January, 2000. Histological findings of erythema showed many carcinoma cells similar to the primary lesion of left breast cancer in the whole dermis. A diagnosis of skin metastasis of breast cancer was made.

These findings suggest that skin metastasis should be taken into account for patients with erythema on the trunk similar to dermatomyositis.



Polymyositis and dermatomyositis associated with malignancy: a 30-year retrospective study.

Wakata N, Kurihara T, Saito E, Kinoshita M.

Fourth Department of Internal Medicine, Toho University, School of Medicine, Tokyo, Japan.


Int J Dermatol 2002 Nov;41(11):729-34 Abstract quote

BACKGROUND: Polymyositis and dermatomyositis in association with malignancy are paraneoplastic syndromes, but the incidence, treatment and factors that predict associated cancer and its prognosis all remain unclear.

PATIENTS AND METHOD: During the 30-year period 1969-99, we treated 64 patients who had polymyositis (including two with cancer) and 28 patients who had dermatomyositis (including 10 with cancer). We compared the clinical findings of the patients who had cancer with the findings of those who did not have cancer.

RESULTS: The risk of cancer is significantly higher in dermatomyositis and somewhat higher in polymyositis. An increased cancer risk was found in male patients with dermatomyositis who were older than 50 years. Cancer was diagnosed within 4 years before or after the diagnosis of polymyositis or dermatomyositis, and usually within 1 year. An operation was not possible in many of the patients with cancer because of the advanced stage of the disease.

CONCLUSION: Our findings suggest that early discovery of malignancy is critical in cases of polymyositis and dermatomyositis.

Routine vs extensive malignancy search for adult dermatomyositis and polymyositis: a study of 40 patients.

Sparsa A, Liozon E, Herrmann F, Ly K, Lebrun V, Soria P, Loustaud-Ratti V, Bouyssou-Gauthier ML, Boulinguez S, Bedane C, Jauberteau MO, Vidal E, Bonnetblanc JM.

Department of Dermatology, University Hospital of Limoges, Limoges, France.


Arch Dermatol 2002 Jul;138(7):885-90 Abstract quote

OBJECTIVE: To identify potential risk factors and the yield of routine screens for early detection of malignancy associated with dermatomyositis (DM) and polymyositis (PM).

DESIGN: Retrospective study of malignancies in all patients with DM or PM followed up between the years 1981 and 2000 and a review of the relationship of DM and PM to malignancy, the usefulness of various tests or examinations for malignancy search, and the patients' course.

SETTING: Departments of internal medicine and dermatology in a teaching hospital.

PATIENTS: Forty consecutive adult patients with DM (33 cases) or PM (7 cases).

MAIN OUTCOME MEASURES: (1) Rate of false-negative results of routine workup and yield (percentage of positive results) of blind malignancy search and (2) comparison of 16 characteristics in patients with malignancy vs those without.

RESULTS: Malignancy occurred in 16 patients: 13 with DM and 3 with PM. In all cases, the diagnosis of malignancy was made concurrently with or shortly after the diagnosis of DM or PM. Factors associated with malignancy were recruitment in the internal medicine department (P =.02), constitutional symptoms (P<.01), a rapid onset of DM or PM (P =.02), the lack of Raynaud phenomenon (P<.01), and a higher mean erythrocyte sedimentation rate (P<.01) and creatine kinase level (P<.01). Initial routine search failed to discover 4 malignancies, 3 of which were discovered at an advanced stage by more extensive investigations. The positive result yield of blind malignancy search was only 13% (11 of 87), but reached 28% (5 of 18) for blind abdominal-pelvic and thoracic computed tomographic scans.

CONCLUSION: Extensive search for malignancy, particularly computed tomographic scans, may be warranted in at least a subset of patients with DM or PM and risk factors of malignancy.

Classical and amyopathic dermatomyositis seen at the National Skin Centre of Singapore: a 3-year retrospective review of their clinical characteristics and association with malignancy.

Ang P, Sugeng MW, Chua SH.

National Skin Centre, Singapore.

Ann Acad Med Singapore 2000 Mar;29(2):219-23 Abstract quote

INTRODUCTION: Dermatomyositis is a condition well-known to medical physicians, especially rheumatologists and dermatologists. Classically, patients present with typical cutaneous features and proximal muscle weakness. Amyopathic dermatomyositis has been reported in recent literature where patients present with skin changes without muscle involvement.

MATERIALS AND METHODS: We reviewed 28 patients diagnosed with dermatomyositis at the National Skin Centre over a 3-year period from 1996 to 1998 to assess the prevalence of this amyopathic variant in our local population and its association with malignancy.

RESULTS: Out of the 28 patients, 13 (46.4%) had no clinical or laboratory evidence of myositis at presentation. Nine patients (32.1%) had clinical muscle weakness and 6 patients (21.4%) had laboratory evidence of myositis. Malignancies were detected in 12 patients (42.8%), half of which were nasopharyngeal carcinomas. There was no significant difference in the prevalence of malignancy between those with detectable muscle weakness (33.3%) and those without (47.4%). However, there was a significant higher prevalence of malignancy in those with clinical and laboratory evidence of myositis (66.6%) than those without (15.4%).

CONCLUSION: We conclude that amyopathic dermatomyositis is a common presentation in our population and may have a lower risk of malignancy than the classical variant. Screening for malignancy, especially nasopharyngeal carcinoma, is recommended for all dermatomyositis patients.

Risk of cancer in patients with dermatomyositis or polymyositis, and follow-up implications: a Scottish population-based cohort study.

Stockton D, Doherty VR, Brewster DH.

Scottish Cancer Intelligence Unit, Information & Statistics Division, Trinity Park House, Edinburgh, EH5 3SQ.

Br J Cancer 2001 Jul 6;85(1):41-5 Abstract quote

We conducted a national, retrospective population-based cohort study of 705 patients hospitalized with a first diagnosis of dermatomyositis (DM) or polymyositis (PM) during 1982-1996 based on linkage of hospital discharge, cancer registration, and death records in Scotland.

Risks of cancer were assessed by calculating standardized incidence ratios (SIR). A first malignancy was diagnosed concurrently or subsequently in 50 patients with DM (SIR 7.7, 95% CI 5.7-10.1), and 40 patients with PM (2.1, 1.5-2.9). Significantly elevated risks were observed for lung, cervix uteri, and ovarian cancer in patients with DM, and for Hodgkin's disease in patients with PM. The excess risk of cancer was highest around the time of diagnosis, and for patients with DM remained high for at least 2 years. Risks were elevated for both sexes but only significantly so for females, and were highest in patients aged 45-74 years at the time of diagnosis for DM and 15-44 for PM.

Incidence of malignant disease in biopsy-proven inflammatory myopathy. A population-based cohort study.

Buchbinder R, Forbes A, Hall S, Dennett X, Giles G.

Department of Clinical Epidemiology, Suite 41, Cabrini Medical Centre, 183 Wattletree Road, Malvern, Victoria, Australia 3144.

Ann Intern Med 2001 Jun 19;134(12):1087-95 Abstract quote

BACKGROUND: The validity and magnitude of an association between myositis and malignant disease continue to be debated. Such issues as the legitimacy of a myositis diagnosis and distinction among myositis subgroups in previous population-based studies remain unresolved.

OBJECTIVE: To determine the risk for malignant disease in patients with biopsy-proven inflammatory myopathies.

DESIGN: Population-based, retrospective cohort study.

SETTING: Victoria, Australia.

PATIENTS: 537 patients in whom a biopsy-positive idiopathic inflammatory myopathy was first diagnosed from 1981 through 1995.

MEASUREMENTS: Standardized incidence ratios were calculated to compare the incidence of malignant disease in patients with inflammatory myopathy and the general population.

RESULTS: A total of 116 cases of malignant disease were found in 104 patients. Seventy-four cases were identified concurrently with (within 7 days) or after diagnosis of myositis. The highest risk for malignant disease was associated with dermatomyositis (standardized incidence ratio, 6.2 [95% CI, 3.9 to 10.0]). The risk was also increased in polymyositis (standardized incidence ratio, 2.0 [CI, 1.4 to 2.7]), although the relative risk for malignant disease in dermatomyositis compared with polymyositis was 2.4 (CI, 1.3 to 4.2). An increased risk for malignant disease was also found in inclusion-body myositis (standardized incidence ratio, 2.4 [CI, 1.2 to 4.9]). The excess risk for malignant disease diminished with time (standardized incidence ratio, 4.4 [CI, 2.7 to 7.1] in the first year; 3.4 [CI, 2.3 to 5.1] between 1 and 3 years; 2.2 [CI, 1.3 to 3.9] between 3 and 5 years; and 1.6 [CI, 1.0 to 2.6] beyond 5 years [ P for trend, 0.002]).

CONCLUSION: The risk for malignant disease is increased in biopsy-proven dermatomyositis and polymyositis and also appears to be increased in inclusion-body myositis.

Predicting factors of malignancy in dermatomyositis and polymyositis: a case-control study.

Chen YJ, Wu CY, Shen JL.

Department of Dermatology, Taichung Veterans General Hospital, no. 160, Sec. 3, Taichung-Kang Road, Taichung, Taiwan China Medical College, Taichung, Taiwan.

Br J Dermatol 2001 Apr;144(4):825-31 Abstract quote

BACKGROUND: An association between dermatomyositis (DM)/polymyositis (PM) and malignancies has been widely reported in the literature. The validity of extensive evaluation for malignancies in those patients has also been questioned for decades. Only limited papers regarding the signs of malignancy and the prognostic factors in DM/PM have been reported.

OBJECTIVES: To define the potential risk factors of concomitant neoplastic diseases in patients diagnosed as having DM/PM.

METHODS: From 1 April 1983 to 30 June 1999, 147 patients were diagnosed as having probable or definite DM/PM at the Veterans General Hospital, Taichung, Taiwan. We excluded four patients who had preceding neoplastic diseases diagnosed before DM/PM, then retrospectively reviewed the data of the remaining 143 patients and subgrouped the cases as four main types: primary idiopathic DM, primary idiopathic PM, juvenile DM/PM and amyopathic DM (ADM). We next performed univariate analysis using logistic regression to evaluate the possible predictive factors for malignancies, such as mean age at onset, gender, manifestations at onset, association with other connective tissue diseases, initial skin presentations, complications and laboratory data. Then we chose the significant factors for multivariate analysis by logistic regression, to determine the independent risk factors of malignancies in DM/PM patients.

RESULTS: Among the 143 patients, DM was the most common type (64%), followed by ADM (14%), juvenile DM/PM (13%) and PM (10%). The mean age at onset overall was 42.4 years. Other connective tissue diseases were present in 22% of all patients, especially PM (50%) and juvenile DM/PM patients (28%). Internal malignancies were present in 13% of patients, and most were associated with DM. Nasopharyngeal carcinomas (NPCs) were the most common tumours. Patients with primary idiopathic DM, with an older age at onset, higher serum creatine phosphokinase levels and male gender, had more chance of developing concomitant malignancies. Those associated with complications, especially interstitial lung diseases, had a lower risk of associated neoplasia. In multivariate analysis, an older age at onset (odds ratio 9.10) and male gender (odds ratio 4.06) were associated with greater risk of developing malignancies.

CONCLUSIONS: The two independent predictive factors for malignancy (P < 0.05) in patients with DM/PM were an older age at onset (> 45 years) and male gender. The primary idiopathic DM group was shown to have higher risk of developing internal malignancies, especially NPC. However, this was not identified as an independent predictive factor for concomitant neoplastic diseases in multivariate analysis. In addition, patients who had the complication of interstitial lung disease had a significantly lower frequency of malignancies (P < 0.001).

Polymyositis and dermatomyositis: short term and longterm outcome, and predictive factors of prognosis.

Marie I, Hachulla E, Hatron PY, Hellot MF, Levesque H, Devulder B, Courtois H.

Department of Internal Medicine, Centre Hospitalier Universitaire de Rouen-Boisguillaume, Rouen, France.

J Rheumatol 2001 Oct;28(10):2230-7 Abstract quote

OBJECTIVE: To assess short term and longterm outcome of polymyositis (PM) and dermatomyositis (DM), and predictive variables of PM/DM course.

METHODS: The medical records of 77 consecutive patients with PM/DM were reviewed. The criteria for PM/DM diagnosis were based upon Bohan and Peter criteria.

RESULTS: Thirty-one patients (40%) achieved remission of PM/DM, whereas 33 (43%) improved and 13 (17%) worsened their clinical status. Short term recurrences of PM/DM (during tapering of therapy) occurred in 36 patients and longterm recurrences (after discontinuation of therapy) in 9 patients. PM/DM were associated with both decreased functional status and quality of life at longterm followup: (1) only 52% of patients considered to be in remission experienced a return to previous normal activities; and (2) 45% of the other patients with nonremitting PM/DM still had a marked reduction of activities (as shown by the disability scale of the Health Assessment Questionnaire). Overall mortality was as high as 22%, and the main causes of death were cancer and lung complications. Factors associated with PM/DM remission were younger age and shorter duration of clinical manifestations prior to therapy initiation. Variables associated with poor outcome of PM/DM were older age, pulmonary and esophageal involvement, and cancer.

CONCLUSION: Our series shows both high morbidity and mortality related to PM/DM, emphasizing that management of PM/DM patients at an early stage is required. Lung complications (i.e., aspiration pneumonia due to PM/DM related esophageal dysfunction and ventilatory insufficiency) were one of the main causes of death in our series, indicating that investigating for subclinical esophageal and lung impairment should become an integral part of initial PM/DM evaluation. The presence of poor prognostic factors should prompt both close followup and aggressive therapy in patients with PM/DM.


Treatment of inflammatory myopathies.

Mastaglia FL, Phillips BA, Zilko P.

Australian Neuromuscular Research Institute and Department of Medicine, Queen Elizabeth II Medical Centre, Perth, Australia.

Muscle Nerve 1997 Jun;20(6):651-64 Abstract quote

The treatment of the immune-mediated inflammatory myopathies remains largely empirical.

Corticosteroids are usually effective in polymyositis and dermatomyositis but may need to be combined with methotrexate or azathioprine in some patients. Intravenous immunoglobulin (IVIg) is effective as add-on therapy in some patients not adequately controlled with steroids or immunosuppressive agents, but further controlled trials of IVIg are necessary to define the indications and optimal dose regimens. Cyclophosphamide, cyclosporin, or chlorambucil may be effective in patients with refractory polymyositis or dermatomyositis. Low-dose whole body or lymphoid irradiation is a last option in severely disabled patients resistant to all other treatments. As a small proportion of patients with inclusion body myositis respond to corticosteroid or immunosuppressive therapy, a 3-6-month trial of such therapy is justified in this condition.

More specific immunotherapy for these disorders awaits identification of the target antigens and further clarification of the immunopathogenetic mechanisms.

Aggressive management of juvenile dermatomyositis results in improved outcome and decreased incidence of calcinosis.

Fisler RE, Liang MG, Fuhlbrigge RC, Yalcindag A, Sundel RP.

Dermatology and Rheumatology Programs, Children's Hospital, Harvard Medical School, Boston, MA 02115, USA.

J Am Acad Dermatol 2002 Oct;47(4):505-11 Abstract quote

BACKGROUND: Long-term consequences of juvenile dermatomyositis (JDM) include onset of calcinosis and subsequent functional impairment. Historic incidence of calcinosis has been reported between approximately 23% and 70%. Recent reports note improved outcome with high-dose steroids, yet the incidence of calcinosis has remained above 30%.

OBJECTIVE: We attempted to determine whether rapid, aggressive disease management can prevent calcinosis and improve functional outcome.

METHODS: Medical records of children with JDM managed at a pediatric medical center during a 10-year period were reviewed to determine (1) interval between onset of symptoms and diagnosis, (2) treatment modality, and (3) functional outcome and presence of calcinosis.

RESULTS: A total of 21 female and 14 male subjects diagnosed with JDM met inclusion criteria, with a mean age of diagnosis of 7.6 +/- 3.9 years. Mean time from onset of symptoms to treatment was 6.6 +/- 8.2 months. Pulse intravenous methylprednisone (30 mg/kg daily) or high-dose prednisone was used in 31 of 35 patients. Patients who failed to respond within 6 weeks were started on a regimen of methotrexate (23/35). At follow-up, 5 patients had mild calcinosis (14%). Onset of calcinosis was associated with a longer time to diagnosis and treatment (30.6 vs 6 months, P =.003), a longer duration of elevated muscle enzymes (34 vs 12.6 months, P =.03), and longer disease duration (42.8 vs 22.2 months, P =.05).

CONCLUSION: Stepwise, aggressive treatment directed at achieving rapid and complete control of muscle inflammation is highly successful in minimizing the long range sequelae of JDM, including calcinosis.

Combination Antimalarials in the Treatment of Cutaneous Dermatomyositis

A Retrospective Study

Gina Charlene Ang, MD; Victoria P. Werth, MD


Arch Dermatol. 2005;141:855-859. Abstract quote

Objective  To observe whether the use of antimalarials in combination resulted in significant improvement in the cutaneous signs and symptoms of patients with dermatomyositis who did not otherwise respond to the use of single-agent antimalarial therapy.

Design  Retrospective case series of 17 patients treated between January 1, 1991, and December 31, 2002.

Setting  An ambulatory medical dermatology clinic in an academic center.

Patients  Patients had adult-onset dermatomyositis with predominantly cutaneous symptoms and a follow-up period at our clinic of at least 6 months. Cases in which it was not possible to assess the effect of treatment on cutaneous symptoms were not included.

Intervention  Treatment regimens varied and included the use of antimalarials, prednisone, methotrexate, and other medications.

Main Outcome Measures  Physician-observed and patient-reported improvement based on erythema, pruritus, and extent of affected skin.

Results  Seven of 17 patients experienced at least near clearance in cutaneous symptoms with the use of antimalarial therapy alone: 4 of these patients required combination therapy (hydroxychloroquine sulfate–quinacrine hydrochloride or chloroquine phosphate–quinacrine), while 3 of them responded well to antimalarial monotherapy. The median time required to reach the response milestones on the final working therapeutic regimen was 3 months (mean, 4.8 months; range, 2-14 months). Six patients did not respond significantly to any type of therapy, including nonantimalarials.

Conclusion  Our experience suggests that a significant subgroup of patients whose skin lesions have been unresponsive to a single antimalarial benefit from combination therapy with hydroxychloroquine and quinacrine or chloroquine and quinacrine, but controlled clinical trials are warranted to assess the extent of benefit.

Adverse cutaneous reactions to hydroxychloroquine are more common in patients with dermatomyositis than in patients with cutaneous lupus erythematosus.

Pelle MT, Callen JP.

310 E Broadway, Louisville, KY 40292.

Arch Dermatol 2002 Sep;138(9):1231-3 Abstract quote

BACKGROUND: Hydroxychloroquine sulfate and other antimalarial drugs have been used successfully as adjunctive therapy for patients with cutaneous lesions of dermatomyositis over the past 20 years. An increased incidence of cutaneous reactions to hydroxychloroquine has been postulated to occur in patients with dermatomyositis.

OBJECTIVE: To determine if adverse cutaneous eruptions due to hydroxychloroquine are more common in patients with dermatomyositis than in those with cutaneous lupus erythematosus.

DESIGN: Retrospective, age-, sex-, and race-matched case-control study.

SETTING: University-affiliated practice.

PATIENTS: The study comprised 42 patients with dermatomyositis (39 adults) and 39 age-, sex-, and race-matched adult patients with lupus erythematosus.

MAIN OUTCOME MEASURES: Presence or absence of documented drug eruption due to hydroxychloroquine exposure.

RESULTS: Of 39 patients, 12 (31%) with dermatomyositis developed a cutaneous reaction to hydroxychloroquine. Among age-, sex-, and race-matched patients with cutaneous lupus erythematosus, only 1 developed a cutaneous reaction to hydroxychloroquine. None of the reactions observed in our patients resulted in serious morbidity or mortality. Additionally, 4 patients with dermatomyositis who reacted to hydroxychloroquine were treated with oral chloroquine phosphate, 2 of whom also reacted to chloroquine phosphate.

CONCLUSIONS: When contemplating antimalarial therapy for dermatomyositis, both the physician and the patient should recognize that non-life-threatening cutaneous reactions may occur in approximately one third of patients and that perhaps one half of those who react to hydroxychloroquine will also react to chloroquine.


Immunoglobulin therapy in inflammatory myopathies.

Mastaglia FL, Phillips BA, Zilko PJ.

Australian Neuromuscular Research Institute, University Department of Medicine, Queen Elizabeth II Medical Centre, Perth WA.

J Neurol Neurosurg Psychiatry 1998 Jul;65(1):107-10 Abstract quote

A prospective open label trial of add on therapy with intravenous immunoglobulin (i.v.Ig) was carried out in 16 patients with inflammatory myopathy who had continued to deteriorate or had relapsed on conventional therapy.

The response was assessed using isometric myometry, functional scales, MRC grading, and serum creatine kinase concentrations with a three month run in period before commencement of i.v.Ig. Five of seven patients with isolated dermatomyositis or polymyositis and all four patients with an overlap syndrome responded to i.v.Ig with partial or complete remission of disease and normalisation of serum creatine kinase concentrations. None of five patients with inclusion body myositis showed any functional improvement although myometry scores improved in some muscles in one case.

It is concluded that i.v.Ig is an effective therapeutic option in patients with drug resistant polymyositis or dermatomyositis. However, further controlled trials are required to confirm the efficacy of this form of treatment and to establish optimal doses and administration regimes.

Controlled studies with high-dose intravenous immunoglobulin in the treatment of dermatomyositis, inclusion body myositis, and polymyositis.

Dalakas MC.

Neuromuscular Diseases Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health Bethesda, MD 20892-1382, USA.

Neurology 1998 Dec;51(6 Suppl 5):S37-45 Abstract quote

There are three major subsets of the inflammatory myopathies: polymyositis (PM), dermatomyositis (DM), and inclusion-body myositis (IBM).

High-dose intravenous immunoglobulin (IVIg) has been tried in controlled clinical trials in patients with DM and IBM but not with PM. In patients with DM that is resistant or partially responsive to conventional therapies, IVIg was very effective. The treated patients experienced dramatic improvement not only in muscle strength but also of their skin rash. Repeated muscle biopsies with quantitative histologic studies showed the IVIg-treated patients had a statistically significant improvement of the muscle cytoarchitecture, with resolution of the aberrant immunopathologic parameters. In two controlled clinical trials conducted in IBM patients, IVIg showed marginal improvements in muscle strength which were nonsignificant. However, a few IBM patients had a definite clinical improvement with increased activities of daily living, but when analyzed within the entire IVIg-treated group, their total gains in muscle strength did not reach statistical significance compared to the placebo-treated group.

Of interest is that certain muscle groups in the IVIg-treated patients, such as the muscles of swallowing, showed significant improvement compared to those of the placebo-treated patients, implying mild regional effects. In PM, uncontrolled trials have shown improvements in muscle strength, but the controlled clinical trial is still ongoing.


Treatment of calcinosis in juvenile dermatomyositis with probenecid: the role of phosphorus metabolism in the development of calcifications.

Harel L, Harel G, Korenreich L, Straussberg R, Amir J.

Department Pediatrics C, Schneider Children's Medical Center of Israel, Petah Tikva.

J Rheumatol 2001 May;28(5):1129-32 Abstract quote

OBJECTIVE: To report the efficacy of probenecid for calcinosis of juvenile dermatomyositis (JDM) and assess the changes in phosphorus metabolism during treatment.

METHODS: Biochemical studies of calcium and phosphorus metabolism were performed in a 9-year-old girl with JDM and extensive calcifications before and during probenecid treatment.

RESULTS: The calcifications resolved over 18 months of treatment. Probenecid was found to be effective in reducing calcifications by increasing renal phosphate clearance.

CONCLUSIONS: The tendency for calcifications in some patients with JDM might be related to an increase in renal phosphate reclamation, and therefore, probenecid treatment may be effective in these patients.

Commonly Used Terms

Gottron's papules-Atrophic papules and plaques over the knuckles.

Heliotrope rash-Purplish discoloration and edema of the periorbital tissues.


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