This is also known as exfoliative dermatitis and the red man syndrome. It is not a specific disease and can be seen in both benign and malignant diseases. Idiopathic (unknown cause) cases may persist longer than other types.
These underlying dermatosis include such diverse conditions as psoriasis, seborrheic dermatitis, atopic dermatitis, pityriasis rubra pilaris, contact dermatitis, photosensitive conditions, pemphigus foliaceus, and bullous pemphigoid. Many drugs can also cause erythroderma, too numerous to list here. Most of these drugs are ingested orally but topical applications may sometimes may be implicated.
In spite of the dramatic clinical appearance the biopsy histopathology is of limited value and dependent upon the underlying condition eliciting the erythroderma. In cases associated cutaneous T-cell lymphomas, the biopsy is usually diagnostic. The pathologist must correlate the histology with the clinical appearance and may give an extensive differential diagnosis.
Epidemiology Disease Associations Pathogenesis Laboratory/Radiologic/Other Diagnostic Testing Gross Appearance and Clinical Variants Histopathological Features and Variants Special Stains/
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EPIDEMIOLOGY CHARACTERIZATION INCIDENCE
The incidence of erythroderma: A survey among all dermatologists in The Netherlands
Vigfús Sigurdsson, MD
Paul H. A. Steegmans, MD
Willem A. van Vloten, MD
Utrecht, The Netherlands
J Am Acad Dermatol 2001;45:675-8. Abstract quote
Background: Erythroderma is a rare skin disorder, and studies on its incidence and causes are lacking. The annual incidence has been estimated to be 1 to 2 patients per 100,000 inhabitants.
Objective: We investigated the incidence and cause of erythroderma in an unselected population and evaluated the referral pattern of erythrodermic patients by nonacademic dermatologists.
Methods: A survey was performed among all nonacademic dermatologists in The Netherlands, using a mailed questionnaire. Questions dealt with the number of patients diagnosed with erythroderma in the year 1997, the cause of the erythroderma, and whether these patients were referred to a university hospital. A questionnaire was also sent to all university hospitals.
Results: Seventy-eight percent of the nonacademic dermatologists answered the questionnaire, and all 8 university hospitals responded. In the year 1997, 141 patients were diagnosed with erythroderma in The Netherlands. The annual incidence of erythroderma based on these figures is 0.9 patients per 100,000 inhabitants. Compared with the university hospitals, erythroderma was more often diagnosed as an exacerbation of preexisting dermatoses (61% vs 51%; P = .37) and less often as idiopathic (14% vs 31%; P = .04) among the nonacademic dermatologists. Cutaneous T-cell lymphoma was rarely the cause, occurring in only 1% of patients diagnosed by the nonacademic dermatologists but in 6% of patients at the university hospitals. Overall, only 37% of nonacademic dermatologists referred patients with erythroderma to a university hospital.
Conclusions: The incidence of erythroderma in The Netherlands is slightly lower than the earlier estimate in the literature. Moreover, the cause of erythroderma is different among patients diagnosed in an academic and a nonacademic setting. The majority of dermatologists in The Netherlands treat patients with erythroderma themselves and do not refer these patients to university hospitals.
DISEASE ASSSOCIATIONS CHARACTERIZATION (Percentage) Underlying dermatosis 62.5% Drug eruptions 16% Idiopathic 15-30% Cutaneous T-cell lymphoma 12.5% GENERAL
Erythroderma. A clinicopathological study of 56 cases.
Botella-Estrada R, Sanmartin O, Oliver V, Febrer I, Aliaga A.
Department of Dermatology, University General Hospital, Valencia, Spain.
Arch Dermatol 1994 Dec;130(12):1503-7 Abstract quote
BACKGROUND AND DESIGN: Erythroderma may be the result of many different causes. There are several publications on this subject, most of them from England, the United States, and the Scandinavian countries reporting a different incidence of each etiologic group. Our objective has been to determine the frequency of erythroderma in our environment, its cause, and patient evolution. We reviewed the clinical, laboratory, and biopsy material of 56 patients diagnosed with erythroderma who were treated in our department in the last 8 years (1984 through 1991). Patients were followed up to know the evolution of the erythroderma.
RESULTS: The male-female ratio was 4:1. The mean age at diagnosis was 57 years. Dermatoses were the most frequent cause of erythroderma (62.5%), followed by topical or systemic drug reactions (16%), and cutaneous T-cell lymphomas (12.5%). Follow-up information was obtained from 42 patients (66%). Eight patients died (19%), but only in three cases was death directly related to erythroderma (7.14%). The group associated with the best prognosis was that related to drugs. The best clinicohistologic correlation was found in cutaneous T-cell lymphoma-related erythroderma.
CONCLUSIONS: Erythroderma of unknown cause and protracted course may be secondary to senile atopic dermatitis, intake of drugs overlooked by the patients, and patients who are in slow progression to cutaneous T-cell lymphoma. Close follow-up of erythrodermas of unknown cause by repeating cutaneous biopsies will in time allow an early diagnosis in patients in the latter group.
Erythroderma: analysis of 247 cases.
Vasconcellos C, Domingues PP, Aoki V, Miyake RK, Sauaia N, Martins JE.
School of Medicine, University of S. Paulo, Brazil.
Rev Saude Publica 1995 Jun;29(3):177-82 Abstract quote
The profile of 247 patients with erythroderma during a 23 year period from January, 1962 through March, 1985, with a follow-up period ranging from 1 to 26 years were analysed. The patients presented with diffuse erythema, scaling and pruritus of more than 2 months' duration, and the age ranged from 16 to 60 years.
Psoriasis was the most frequent underlying disease with an estimated frequency of 44.9%, the reaction to the use of drugs appeared in 7.3% of total cases and association with reticulosis showed a frequency of 4.1%. The cause of the erythroderma could not be determined in 29.2% of the cases. Six differences in terms of underlying diseases were not observed. One or more skin biopsies along with clinical findings were diagnostic or suggestive of the underlying disease in 63.6% of the cases.
Repeated skin biopsies are recommended as the best method for etiologic diagnosis of erythroderma. At P = 0.05 significance level, masculine/feminine ratio of 2:1 was found. The question arises whether causal agent of erythroderma may not be somehow related to different exposure by sex to environmental antigens.
Transient eruptive seborrheic keratoses associated with erythrodermic psoriasis and erythrodermic drug eruption: Report of two cases
Scott L. Flugman, MD
Steve A. McClain, MD
Richard A. F. Clark, MD
Stony Brook, New York
J Am Acad Dermatol 2001;45:S212-4 Abstract quote
The appearance of multiple seborrheic keratoses in association with underlying internal malignancy (known as the sign of Leser-Trelat) has generated much discussion and debate in the literature. However, comparatively few case reports exist that examine the appearance of multiple seborrheic keratoses associated with exfoliative erythroderma without underlying malignancy.
We report 2 cases in which multiple, biopsy-proven seborrheic keratoses appeared in conjunction with erythrodermic skin eruptions. The underlying diseases in these 2 patients included psoriasis and an eczematous drug eruption; in both cases the erythroderma resolved with appropriate treatment. After resolution of the erythroderma, the newly developed seborrheic keratoses proceeded to involute and gradually fall off. Neither of the patients exhibited any evidence of internal malignancy.
These cases represent the first reports of psoriasis and drug eruption as causes of erythroderma-induced transient eruptive seborrheic keratoses. Clinical and pathologic findings are consistent with previous descriptions of this entity.
OTHER ASSOCIATIONS HIV
Erythroderma: a comparison between HIV positive and negative patients.
Morar N, Dlova N, Gupta AK, Naidoo DK, Aboobaker J, Ramdial PK.
Dermatology Department, Faculty of Medicine, University of Natal and King Edward VII Hospital, Congella, South Africa.
Int J Dermatol 1999 Dec;38(12):895-900 Abstract quote
Erythroderma has protean underlying causes. There have been isolated case reports suggesting an association between erythroderma and the human immunodeficiency virus (HIV).
OBJECTIVE: To describe and characterize further the prevalence, etiology, and metabolic sequelae of erythroderma in HIV positive and negative patients. In a subset of patients, clinicopathologic correlation was performed.
METHOD: One hundred and thirty-eight consecutive patients were prospectively recruited over a one and a half year period at the skin clinic of King Edward VIII Hospital. Demographic, clinical, biochemical, and histologic data were recorded.
RESULTS: Seventy-five per cent of the patients were black, 22.5% Indian, and 2.5% white. The men to women ratio was 1.9 : 1. The mean age was 34. 7 years (range, 1 month to 85 years). Forty-three per cent of patients were HIV positive, of whom 90% were black. The commonest causes of erythroderma in the total sample were atopic dermatitis (23.9%), psoriasis (23.9%), and drug reactions (22.5%). The commonest cause in the HIV positive group was drug reactions (40.6%), the commonest being ethambutol (30.8%). HIV positive patients had a significantly lower (P < 0.05) white cell count (7.6 vs. 10.5 x 109 /L), hemoglobin (11.1 vs. 12.6 g/dL), platelets (278.3 vs. 378.0 x 109 /L), and albumin (25.4 vs. 28.7 g/L) and significantly higher serum urates (0.6 vs. 0.4 mM/L) than HIV negative patients. HIV positive patients did not have a significant increase in the number of episodes of erythroderma. Clinicopathologic correlation was greatest with psoriasis in the HIV negative group and with psoriasis and drug reactions in the HIV positive group.
CONCLUSIONS: A large proportion of erythrodermic patients in this study were HIV positive. Inflammatory dermatoses were the commonest cause of erythroderma in all the patients studied. Drug reactions were the commonest cause in HIV positive patients. In the young black patient, erythroderma may be a marker for HIV infection.
PATHOGENESIS CHARACTERIZATION SQUAMOUS CELL CARCINOMA ANTIGENS
Immunohistochemical study of elevated expression of squamous cell carcinoma (SCC)-related antigens in erythrodermic epidermis.
Horiuchi Y, Tsukahara T, Otoyama K.
Division of Dermatology, Kashima Rosai Hospital, Japan.
J Dermatol 1994 Feb;21(2):67-72 Abstract quote
Using immunohistochemical staining with the monoclonal antibodies (mAb) of squamous cell carcinoma-related antigens (SCC-RAg), the expression of SCC-RAg in erythrodermic epidermis, which included senile erythroderma following eczema, atopic erythroderma, psoriatic erythroderma, and Sezary syndrome was examined.
In senile erythroderma and atopic dermatitis, the most intense staining with SCC-RAg mAb was evident in the upper epidermis. The cytoplasm of epidermal cells showed particularly strong staining. Although serum SCC-RAg in the Sezary syndrome was not as high as in other erythroderma patients, staining with SCC-RAg mAb was relatively strong in the upper epidermis. However, in psoriatic erythroderma cases with severe skin conditions, staining was weakly positive and diffuse throughout the entire epidermis.
Following treatment, SCC-RAg decreased significantly with remission. Elevated SCC-RAg release may be attributed to epidermal cells, and SCC-RAg should prove useful as a clinical marker of erythroderma.
CHARACTERIZATION POLYMERASE CHAIN REACTION
- Usefulness of cutaneous T-cell clonality analysis for the diagnosis of cutaneous T-cell lymphoma in patients with erythroderma.
Cordel N, Lenormand B, Courville P, Helot MF, Benichou J, Joly P; French Study Group on Cutaneous Lymphomas.
Department of Dermatology, INSERM Unit, Charles Nicolle Hospital, Rouen, France.
Arch Pathol Lab Med. 2005 Mar;129(3):372-6. Abstract quote
CONTEXT: Demonstration of a dominant T-cell clone in skin biopsy specimens by a molecular assay constitutes an additional diagnostic criterion to differentiate cutaneous T-cell lymphomas (CTCLs) from inflammatory dermatoses.
OBJECTIVE: To determine which patients, depending on their clinical presentations, could most benefit from a cutaneous T-cell clonality analysis in addition to histopathologic analysis for the diagnosis of CTCL.
DESIGN: Comparison of sensitivity and specificity of histopathologic analysis and a combination of this method and the detection of a T-cell receptor gamma chain gene rearrangement by polymerase chain reaction denaturing gradient gel electrophoresis performed on skin biopsy specimens obtained at initial presentation.
PATIENTS: One hundred forty consecutive patients were classified into 4 groups, depending on their clinical presentation: (1) eczematous patches suggestive of early-stage mycosis fungoides (MF) (IA and IB of the TNM classification) (n = 42); (2) plaques, nodules, or tumors that arise on or are associated with plaques suggestive of late-stage MF (IIB and III of the TNM classification) (n = 16); (3) erythroderma (n = 50); and (4) nodules or tumors that arise in normal skin, suggestive of non-MF CTCL (n = 32).
RESULTS: When compared with histopathologic examination, the addition of clonality analysis increased the sensitivity of CTCL diagnosis in all groups of patients except those with cutaneous lesions suggestive of late-stage MF, because the diagnosis was made based on histopathologic analysis alone in 100% of these cases. The main increase in sensitivity of CTCL diagnosis was observed in patients with erythroderma: 62% with histopathologic analysis alone to 87% with the combination of both methods (P = .04). Diagnostic specificity of molecular assays decreased from 100% to 76% (P = .01) in patients with patch lesions and from 100% to 70% (P = .04) in patients with nodules that occurred in normal skin due to the detection of a T-cell clone in 6 patients with follicular mucinosis without a histologic pattern of MF and in 5 of 20 cases of T-cell pseudolymphoma (25%), respectively. In contrast, a T-cell clone was not detected in the 34 patients with erythroderma of inflammatory origin.
CONCLUSION: Polymerase chain reaction analysis of cutaneous T-cell clonality could be useful for the diagnosis of CTCL in patients who present with erythroderma.
Heteroduplex analysis of T-cell receptor gamma gene rearrangement as an adjuvant diagnostic tool in skin biopsies for erythroderma.
Cherny S, Mraz S, Su L, Harvell J, Kohler S.
Department of Pathology, Stanford University Medical Center, Stanford California 94305, USA.
J Cutan Pathol 2001 Aug;28(7):351-5 Abstract quote
BACKGROUND: Erythroderma, defined as red skin covering most of the body surface often accompanied or followed by exfoliation, is the clinical manifestation of at least six different underlying etiologies with allergic or irritant contact dermatitis, atopic/asteotic dermatitis, pityriasis rubra pilaris (PRP), psoriasis, and seborrheic dermatitis accounting for the majority of cases. Approximately 10% of cases are due to adverse drug reactions with roughly another 10% due to cutaneous T-cell lymphoma (CTCL), predominantly mycosis fungoides, or leukemia. It is clear from multiple studies that the clinical diagnosis of the underlying entity is often difficult, as these diseases can present in a very similar fashion. A skin biopsy is usually employed in this setting as a diagnostic tool. However, the histopathologic diagnosis of the underlying cause is complicated by the subtlety of the distinguishing histologic features. In this situation, an ancillary technique demonstrating the presence of a monoclonal T-cell proliferation could help to rule in or out CTCL in cases that clinically and histopathologically do not allow a definitive diagnosis.
METHODS: We retrospectively studied 25 biopsies from sixteen patients who presented to the Stanford Dermatology Clinic with erythroderma. We examined the specimens morphologically and analyzed the gamma chain of the T-cell receptor (TCR- gamma) by polymerase chain reaction (PCR) followed by heteroduplex analysis for clonality. We then correlated the results of our PCR and heteroduplex analyses with the patients' clinical outcomes.
RESULTS: Four biopsies, from three patients, contained clonal TCR-gamma rearrangements; the four biopsies, all of which were equivocal histologically, correlated to diagnoses of mycosis fungoides (MF) or Sezary syndrome (SS). Twenty-one biopsies contained polyclonal T-cell populations. Eighteen of these biopsies represent patients with inflammatory dermatoses. Three of these biopsies, all of which were taken from a single patient, correlate to a diagnosis of MF.
CONCLUSION: TCR-gamma PCR heteroduplex analysis seems to represent an important adjuvant diagnostic tool that, used in conjunction with histopathology and clinical history, could help to clarify the underlying etiology of erythroderma.
CLINICAL VARIANTS CHARACTERIZATION PEDIATRIC
Erythroderma in children: a clinico-etiological study.
Sarkar R, Sharma RC, Koranne RV, Sardana K.
Department of Dermatology and Sexually Transmitted Diseases, Lady Hardinge Medical College, New Delhi, India.
J Dermatol 1999 Aug;26(8):507-11 Abstract quote
Although there are various published studies on erythroderma from western and Asian countries, most of them have only included patients in the adult age groups. As we have an exclusively pediatric dermatology unit, we thought it would be intriguing to study the clinical, etiological and laboratory parameters of erythroderma in children. Seventeen erythroderma patients of both sexes were inducted into the study between 1993 to 1998.
The mean age of onset was 3.3 years and the male:female ratio was 0.89:1. Eight (47%) of the patients were infants; 9 (53%) others belonged to the preschool and school going age group (age range between 1 to 12 years). An acute onset of the disease was seen in 47% of the patients while 53% of the patients had a chronic onset. The main presenting complaints were itching in 41% and burning in 18% of patients. Scalp involvement (71%), nail involvement (18%), and alopecia (6%) were the main cutaneous features observed while fever (53%), tachycardia (53%), pedal edema (12%), lymphadenopathy (18%), and hepatomegaly (12%) were the main systemic features observed in this study. Etiologically, drugs (29%), showed the highest incidence, followed equally (18%) by genodermatoses, psoriasis, and staphylococcal scalded skin syndrome (SSSS). Two (12%) patients had erythroderma due to atopic dermatitis, while one was (5%) due to infantile seborrheic dermatitis coexisting with dermatophytosis. Laboratory parameters contributed little towards diagnosis of the underlying dermatological condition.
Thus, though erythroderma is a striking entity, it is yet uncommon in the pediatric age group. Because the drug induced group was the largest in this study, we recommend that drugs should be suspected as important causative factors of erythroderma in children.
HISTOPATHOLOGY CHARACTERIZATION GENERAL
The specificity of histopathology in erythroderma.
Zip C, Murray S, Walsh NM.
Department of Medicine, Victoria General Hospital, Halifax, Nova Scotia, Canada.
J Cutan Pathol 1993 Oct;20(5):393-8 Abstract quote
Conflicting views about the diagnostic value of skin biopsy in the investigation of erythrodermic patients are extant. The objective of the present study was to establish the frequency with which a correct diagnosis can be based on histopathological assessment alone. This was achieved by comparison of the "blinded" microscopic diagnosis with the final diagnosis (based on combined clinico-pathologic parameters and response to therapy).
In a retrospective review of 56 skin biopsies from 40 patients with erythroderma, we found a positive correlation between the pathological diagnosis and the final diagnosis in 66%; furthermore, when the microscopic characteristics observed in different diagnostic categories were assessed, these proved to simulate those seen in conventional manifestations of the various underlying diseases but tended to be subtle in the setting of erythroderma.
We conclude that, despite the homogeneity of the clinical expression of erythroderma, diagnostic histopathological features of the underlying disease are retained in the majority of cases.
Histopathology in erythroderma: review of a series of cases by multiple observers.
Walsh NM, Prokopetz R, Tron VA, Sawyer DM, Watters AK, Murray S, Zip C.
Department of Pathology, Victoria General Hospital, Halifax, Nova Scotia.
J Cutan Pathol 1994 Oct;21(5):419-23 Abstract quote
This study examines the utility of objective histopathological studies in the evaluation of adult patients with erythroderma. A series of 56 skin biopsies, from 40 erythrodermic patients, was reviewed sequentially by 4 Canadian dermatopathologists who were unaware of clinical details of the cases.
The final diagnosis (gold standard), in each instance, had already been determined by others, based on clinicopathologic data and response to therapy. Direct comparison revealed that the mean accuracy of the histopathological diagnoses was 53% (range: 48-66%), a favorable result in view of the difficulty of the task at hand.
Additional points of information which evolved from the study are as follows: (i) identification, by microscopy alone, of spongiotic dermatitis, cutaneous T-cell lymphoma and psoriasis, as underlying causes of erythroderma was more successful than that of drug eruptions and pityriasis rubra pilaris; (ii) the epidermotropism which characterizes cutaneous T-cell lymphoma may be mistaken for inflammatory interface changes seen in drug eruptions and vice versa, thus constituting a pitfall in diagnosis; (iii) finally, it appears that submission of multiple simultaneous biopsies, rather than a single specimen, from patients with erythroderma would be likely to enhance the accuracy of histopathological diagnosis.
Psoriatic erythroderma: a histopathologic study of forty-five patients.
Tomasini C, Aloi F, Solaroli C, Pippione M.
Department of Dermatology, University of Turin, Italy.
Dermatology 1997;194(2):102-6 Abstract quote
BACKGROUND: There are conflicting opinions about the diagnostic value of skin biopsy in erythrodermic psoriasis.
OBJECTIVE: The purpose of the present study was to establish the specificity of the histopathologic changes of psoriatic erythroderma.
METHODS: We reviewed 52 skin biopsies from 45 erythrodermic patients having a final diagnosis of psoriasis on the basis of combined clinical and laboratory data, in addition to response to therapy and follow-up. In 5 patients, erythroderma was the presenting sign of psoriasis. A control group of nonpsoriatic erythrodermic patients was also included in the study.
RESULTS: Among the group of patients with a discharge diagnosis of psoriatic erythroderma, the histopathologic changes were specific for psoriasis in 40 cases (88%). The changes of early macular and squamous lesions of psoriasis were more often found in the biopsy specimens of our series than those of fully developed or late lesions of psoriasis. They included mainly slight epidermal hyperplasia, focal disappearance of the granular layer, mounds of parakeratosis and extravasated erythrocytes within edematous dermal papillae associated with perivascular and interstitial infiltration of lymphocytes and histiocytes.
CONCLUSION: When features of early lesions of psoriasis are found during the evaluation of a biopsy specimen from a patient with a clinically nonspecific erythroderma, the dermatopathologist should be aware that this patient could have psoriasis and a renewed anamnesis and a close follow-up should be made.
- Erythroderma with lichenoid granulomatous features induced by erythropoietin.
Wolf IH, Smolle J, Cerroni L, Kerl H.
Department of Dermatology, University of Graz, Graz, Austria.
J Cutan Pathol. 2005 May;32(5):371-4. Abstract quote
The increasing use of new drugs in cancer therapy, especially growth factors, hormones, and chemotherapies resulted in several reports of unusual skin eruptions.
We studied a patient with erythroderma who had received erythropoietin because of myeloma with tumor anemia. The histological features were characterized by a lichenoid, focally granulomatous infiltrate with predominance of histiocytes.
It is important for dermatopathologists to recognize this interesting pattern induced by erythropoietin.
DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES
Histopathologic studies in Sezary syndrome and erythrodermic mycosis fungoides: a comparison with benign forms of erythroderma.
Sentis HJ, Willemze R, Scheffer E.
J Am Acad Dermatol 1986 Dec;15(6):1217-26 Abstract quote
Histologic sections from eleven patients with Sezary syndrome were reviewed and compared with those of four patients with erythrodermic mycosis fungoides and twenty-four patients with a benign form of erythroderma, including fifteen patients with chronic dermatitis, four with a generalized drug eruption, and five with an erythrodermic psoriasis.
The most important discriminating histologic feature in patients with Sezary syndrome was the presence of a monotonous bandlike or perivascular infiltrate in the papillary dermis, mainly composed of large cerebriform-mononuclear cells, as seen in seven of eleven Sezary syndrome patients. Pautrier's microabscesses were observed in seven of eleven Sezary syndrome patients, two of four patients with erythrodermic mycosis fungoides, but not in any of the patients with a benign form of erythroderma; their presence was therefore considered a reliable criterion in differentiating erythrodermic cutaneous T cell lymphoma from benign forms of erythroderma. However, features of chronic dermatitis were often found superimposed on those of Sezary syndrome and were even predominating in four of eleven Sezary syndrome patients. Moreover, four patients with a benign form of erythroderma showed a histologic picture suggestive of cutaneous T cell lymphoma.
Therefore, in dubious cases repeated skin biopsies, additional investigations of lymph nodes and peripheral blood, and careful follow-up are mandatory for the achievement of a correct diagnosis.
PROGNOSIS AND TREATMENT CHARACTERIZATION PROGNOSIS
Erythroderma: a follow-up of fifty cases.
Hasan T, Jansen CT.
J Am Acad Dermatol 1983 Jun;8(6):836-40 Abstract quote
Fifty consecutive cases of erythroderma were studied. The mean onset age was 61 years, and thirty-three of the fifty were male. As a causative factor, a preexisting dermatosis was identified in twenty-one cases, topical sensitization to drugs in six cases, reaction to internal drugs in five cases, and mycosis fungoides in two cases. In sixteen cases a causative factor could not be disclosed.
A follow-up survey of the patients at a mean of 6 years after the start of the erythroderma indicated that seventeen patients had cleared completely, twelve patients had less symptoms than initially, and six suffered from undiminished erythroderma. Ten patients had died from causes unrelated to the erythroderma.
It is concluded that the underlying causes for erythroderma have remained rather unchanged during several decades, and that the majority of present-day erythroderma patients can expect a favorable prognosis.
Idiopathic erythroderma: a follow-up study of 28 patients.
Sigurdsson V, Toonstra J, van Vloten WA.
Department of Dermatology, University Hospital, Utrecht, the Netherlands
Dermatology 1997;194(2):98-101 Abstract quote
BACKGROUND: Erythroderma may result from different causes, but a proportion remains undetermined (idiopathic erythroderma). Patients with idiopathic erythroderma have often been regarded to have a pre-Sezary syndrome because some of these patients have developed a cutaneous T-cell lymphoma during follow-up.
OBJECTIVE: The aim of this study was to investigate if this was true for our group and also if it is possible to identify further which patients are at high risk of developing cutaneous T-cell lymphoma.
METHODS: We analyzed clinical and follow-up data and reviewed the skin histopathology of all patients who were diagnosed with idiopathic erythroderma in our clinic between 1977 and 1994.
RESULTS: Twenty-eight patients, 16 males and 12 females, were diagnosed with idiopathic erythroderma. This is 27% of the patients who were diagnosed with erythroderma in our clinic, during this period. During the median follow-up of 33 months, 35% of the patients went into complete remission and 52% showed partial remission. Three patients (13%), all females, had persistent chronic erythroderma. Two of the latter group progressed to cutaneous T-cell lymphoma, i.e. 1 to Sezary syndrome and 1 to mycosis fungoides.
CONCLUSION: Based on our results we conclude that only patients with persistent chronic idiopathic erythroderma, which is a minority, have an increased risk of developing cutaneous T-cell lymphoma and therefore need a close and long-term follow-up.
TREATMENT Depends upon underlying disease
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