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Background
This disorder, also known as REM, is a dermal mucinosis presenting as erythematous infiltrated plaques with a reticulated appearance. It characteristically occurs on the midline of the back and chest and may spread to the upper abdomen. The condtion may be exacerbated by sunlight and may last for many years. Skin biopsy shows a superficial to mid-dermal perivascular lymphocytic infiltrate associated with dermal mucinosis. There is no proliferation of fibroblasts.
OUTLINE
EPIDEMIOLOGY CHARACTERIZATION May be more common in women
DISEASE ASSOCIATIONS CHARACTERIZATION CARCINOMA, LUNG
- Reticular erythematous mucinosis (REM) with telangiectasias associated with essential thrombocytosis and lung carcinoma.
Leon-Mateos A, Ginarte M, Leon L, Toribio J.
Department of Dermatology Clinical University Hospital Faculty of Medicine. C/ San Francisco s/n, 15782 Santiago de Compostela, Spain.
Eur J Dermatol. 2005 May-Jun;15(3):179-81. Abstract quote
The cutaneous mucinoses are a heterogeneous group of diseases in which mucin accumulates in the skin. Reticular erythematous mucinosis (REM) is an infrequent variant.
We present a 48-year-old man with essential thrombocytosis and REM lesions with atypical telangiectasias on his chest, who developed a non-small cell lung carcinoma.
We discuss the unusual clinical finding of telangiectasias over REM lesions and the association with essential thrombocytosis and lung carcinoma.HASHIMOTO'S THYROIDITIS Reticular erythematous mucinosis and acral papulokeratotic lesions associated with myxoedema due to Hashimoto thyroiditis.
Velasco JA, Santos JC, Villabona V, Santana J.
Department of Dermatology, General University Hospital, University of Salamanca, Spain.
Dermatology 1992;184(1):73-7 Abstract quote
A case is described of a patient with generalized myxoedema due to Hashimoto thyroiditis involving lesions of reticular erythematous mucinosis and acral papulokeratotic lesions with 'church spire' histological pattern. Substitutive treatment with thyroid hormone led to a rapid regression of the cutaneous lesions.
The infrequent association of reticular erythematous mucinosis lesions and other forms of cutaneous mucinoses with hypothyroidism is discussed, with special emphasis on the fact that the underlying thyroid disease in all cases was Hashimoto thyroiditis.
The association of distal keratotic papules with hypothyroidism has not been previously reported in the literature.
HIV INFECTION Reticular erythematous mucinosis associated with human immunodeficiency virus infection.
Dauden E, Penas PF, Buezo GF, Fraga J, Garcia-Diez A.
Department of Dermatology, Hospital de la Princesa, Universidad Autonoma, Madrid, Spain.
Dermatology 1995;191(2):157-60 Abstract quote
We report the case of a 56-year-old woman with reticular erythematous mucinosis (REM).
During her workup infection with the human immunodeficiency virus (HIV) was detected. She developed a cerebral toxoplasmosis, salmonella sp. bacteremia and oral ulcerations with the presence of type I herpes simplex virus and cytomegalovirus. The relation of REM with the deposition of mucin in AIDS patients' bone marrow and HIV infection is discussed.
To our knowledge, this is the first report where REM is associated with HIV disease.
IDIOPATHIC THROMBOCYTOPENIC PURPURA Reticular erythematous mucinosis and thrombocytopenic purpura. Report of a case and review of the world literature, including plaquelike cutaneous mucinosis.
Braddock SW, Davis CS, Davis RB.
Department of Internal Medicine, University of Nebraska Medical Center, Omaha 68105-1065.
J Am Acad Dermatol 1988 Nov;19(5 Pt 1):859-68 Abstract quote
A case of reticular erythematous mucinosis associated with chronic idiopathic thrombocytopenic purpura and circulating immune complexes is described.
We compare reticular erythematous mucinosis with the similar plaquelike cutaneous mucinosis. We discuss the apparent photosensitivity of reticular erythematous mucinosis and its possible relationship with altered states of immune function such as diabetes, thyroid disease, and neoplasia. The somewhat varied but characteristic histopathologic findings and staining are reviewed, including the characteristic perivascular and occasional perifollicular lymphocytic infiltrate and Alcian blue-positive dermal mucin.
Treatment with antimalarial drugs still appears to be the most uniformly successful therapeutic approach to the management of this chronic dermatosis. Further research efforts should be directed at understanding its link with altered states of immune function.
MYCOSIS FUNGOIDES
- Mycosis fungoides presenting as reticular erythematous mucinosis.
Twersky JM, Mutasim DF.
Department of Dermatology, University of Cincinnati College of Medicine, OH 45267, USA.
Int J Dermatol. 2006 Mar;45(3):230-3. Abstract quote
BACKGROUND: Mycosis fungoides has numerous clinical presentations.
METHODS: We present two cases in which mycosis fungoides clinically mimicked reticular erythematous mucinosis.
RESULTS: Both patients had extracutaneous involvement. One had a fatal outcome.
CONCLUSIONS: We suggest that full systemic evaluation be performed at the time of presentation of patients with this variant of mycosis fungoides.
PATHOGENESIS CHARACTERIZATION DERMAL DENDROCYTES Reticular erythematous mucinosis syndrome with an infiltration of factor XIIIa+ and hyaluronan synthase 2+ dermal dendrocytes.
Tominaga A, Tajima S, Ishibashi A, Kimata K.
Department of Dermatology, National Defense Medical College, 3-2 Namiki, Tokorozawa, Saitama 359-8513, Japan.
Br J Dermatol 2001 Jul;145(1):141-5 Abstract quote
We report a patient with reticular erythematous mucinosis (REM) syndrome.
Content of hyaluronan in lesional skin was approximately 2.9-fold higher than in the patient's uninvolved skin, but its synthetic activity in fibroblasts explanted from lesional skin remained unchanged.
Immunohistochemical study using antifactor XIIIa (anti-FXIIIa) antibody demonstrated that the number of FXIIIa+ cells in the lesional skin was significantly increased compared with those in the patient's uninvolved skin and in normal control skin samples (P < 0.01). As hyaluronan is considered to be synthesized by hyaluronan synthase (HAS), which is composed of three genetically distinct isoforms (HAS1, HAS2 and HAS3), the cells responsible for the accumulation of hyaluronan in lesional skin were immunohistochemically examined using antibodies for HAS1, HAS2 and HAS3. The specific antibody for HAS2 was found to react with some populations of FXIIIa+ cells in the involved skin, and the number of HAS2+ cells was significantly increased in the involved skin (P < 0.01).
The results suggest that accumulation of hyaluronan in REM may be related to populations of FXIIIa+/HAS2+ dermal dendrocytes rather than to dermal fibroblasts.
GLYCOS-AMINOGLYCAN SYNTHESIS Reticular erythematous mucinosis syndrome: glycosaminoglycan synthesis by fibroblasts and abnormal response to interleukin-1 beta.
Izumi T, Tajima S, Harada R, Nishikawa T.
Department of Dermatology, Keio University School of Medicine, Tokyo, Japan.
Dermatology 1996;192(1):41-5 Abstract quote
BACKGROUND: Reticular erythematous mucinosis (REM) syndrome is characterized by an accumulation of mucin in the dermis.
OBJECTIVE: To elucidate the mechanism of mucin deposition, the response of REM syndrome fibroblasts to various exogenous factors was studied.
METHOD: REM and control fibroblasts were treated with potent modulators including tumor necrosis factor-alpha, basic fibroblast growth factor, interferon-gamma, transforming growth factor-beta 1 and interleukin-1 alpha (IL-1 alpha) and -4.
RESULTS: IL-1 beta failed to stimulate glycosaminoglycan synthesis in REM fibroblasts whereas it stimulated glycosaminoglycan synthesis up to 6-fold in control fibroblasts. The stimulation of glycosaminoglycan was caused largely by a comparable increase in hyaluronic acid.
CONCLUSION: These results suggest that the patient fibroblasts exhibit an abnormal response to stimulation by exogenous IL-1 beta and that IL- 1 beta may be involved in the abnormal hyaluronic acid metabolism in REM syndrome.
GROSS APPEARANCE/
CLINICAL VARIANTSCHARACTERIZATION GENERAL VARIANTS PEDIATRIC Reticular erythematous mucinosis syndrome: review of the world literature and report of the syndrome in a prepubertal child.
Cohen PR, Rabinowitz AD, Ruszkowski AM, DeLeo VA.
Department of Dermatology, College of Physicians and Surgeons, Columbia University, New York.
Pediatr Dermatol 1990 Mar;7(1):1-10 Abstract quote
An 8-year-old boy with reticular erythematous mucinosis syndrome had erythematous plaques on his chest, face, and arms for three years. Sun exposure resulted in pruritus and increased lesions.
Histologic examination revealed a perivascular mononuclear cell infiltrate with hematoxylin and eosin staining, positive staining material between the dermal collagen bundles with alcian blue (pH 2.5) staining, and granular basement membrane deposits of IgM with direct immunofluorescence staining. Results of all lupus erythematosus serologies and porphyrin studies were negative. Minimal erythema dose determinations to ultraviolet A and B were normal, and the lesions could not be induced with high doses of irradiation.
Topical sunscreens, corticosteroid cream, and systemic beta-carotene produced no therapeutic benefit.
PLAQUE-LIKE Plaquelike cutaneous mucinosis: its relationship to reticular erythematous mucinosis.
Quimby SR, Perry HO.
J Am Acad Dermatol 1982 May;6(5):856-61 Abstract quote
Nine patients with plaquelike cutaneous mucinosis, including the three described originally in 1960, are presented.
The clinical manifestations of asymptomatic persistent, erythematous, infiltrated papules, either isolated or coalescing into plaques, in the midline of the back or chest are suggestive of the diagnosis. The dermal histopathologic findings of perivascular and periappendageal round cell infiltrates and mucin (acid mucopolysaccharides) on special stains confirm the diagnosis.
This syndrome is more common among women, may be aggravated by exposure to ultraviolet light, and may subside spontaneously after long periods.
We believe the condition of these patients is identical to that described as reticular erythematous mucinosis syndrome.
PSORIASIFORM LESIONS Reticular erythematous mucinosis syndrome associated with psoriasis-like eruptions.
Izumi T, Tajima S.
Department of Dermatology, Keio University School of Medicine, Tokyo, Japan.
J Dermatol 1995 Sep;22(9):700-3 Abstract quote
We report a case of reticular erythematous mucinosis syndrome associated with psoriasis-like eruptions. Although the two skin diseases are clinically distinct, they may be related in some ways.
HISTOLOGICAL TYPES CHARACTERIZATION GENERAL Reticular erythematous mucinosis: light and electron microscopy, immunofluorescence and histochemical findings.
Bleehen SS, Slater DN, Mahood J, Church RE.
Br J Dermatol 1982 Jan;106(1):9-18 Abstract quote
Five patients with reticular erythematous mucinosis were studied by skin biopsy and phototesting.
Light microscopy showed a dermal mononuclear cell infiltrate (composed largely of lymphocytes), separation of collagen bundles and fragmentation of elastic fibres. Histochemical stains showed increased dermal mucin with a profile consistent with hyaluronic acid.
In one case, the ultrastructural distribution of mucin was demonstrated using colloidal iron staining, electron microscopy and X-ray microanalysis.
In two cases, virus-like tubular aggregates were identified in the cytoplasm of dermal endothelial cells and pericytes. Immunofluorescent staining for immunoglobulins, fibrin and complement was negative.
SPECIAL STAINS/
IMMUNOPEROXIDASE/
OTHERCHARACTERIZATION SPECIAL STAINS IMMUNOPEROXIDASE Clinical and immunologic studies in reticular erythematous mucinosis and Jessner's lymphocytic infiltrate of skin.
Braddock SW, Kay HD, Maennle D, McDonald TL, Pirruccello SJ, Masih A, Klassen LW, Sawka AR.
Department of Internal Medicine, University of Nebraska Medical Center, Omaha 68198-4360.
J Am Acad Dermatol 1993 May;28(5 Pt 1):691-5 Abstract quote
BACKGROUND: Little is understood about reticular erythematous mucinosis and Jessner's lymphocytic infiltrate of skin.
OBJECTIVE: Our purpose was to define reticular erythematous mucinosis and Jessner's lymphocytic infiltrate of skin further with focus on immunologic studies.
METHODS: In patients with reticular erythematous mucinosis and Jessner's lymphocytic infiltrate of skin, we measured circulating immune complexes before, during, and after therapy. We examined natural killer cells in a functional assay; we performed direct immunofluorescence and T- and B-cell marker studies in skin biopsy specimens.
RESULTS: The infiltrate in reticular erythematous mucinosis is composed of helper T cells. Circulating immune complexes are increased in both reticular erythematous mucinosis and Jessner's lymphocytic infiltrate of skin and decrease with hydroxychloroquine therapy and clinical clearing. Natural killer cell function is decreased in reticular erythematous mucinosis and Jessner's lymphocytic infiltrate of skin.
CONCLUSION: Changes in circulating immune complex titers accompanying therapy with hydroxychloroquine and clinical clearing, with recurrence of the condition and increase in circulating immune complexes on discontinuation of treatment, point to a possible relation between these events.
DIRECT IMMUNO-FLUORESCENCE Reticular erythematous mucinosis syndrome. Report of a case with positive immunofluorescence.
Del Pozo J, Martinez W, Almagro M, Yebra MT, Garcia-Silva J, Fonseca E.
Department of Dermatology, Hospital Juan Canalejo, La Coruna, Spain.
Clin Exp Dermatol 1997 Sep;22(5):234-6 Abstract quote
We report a case with the clinical and histological features of the reticular erythematous mucinosis syndrome (REM), in which there was moderate, continuous, fine, granular, IgM deposition along the basal layer.
Similar direct immunofluorescence results have been reported in only two previous cases.
DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES DERMAL MUCINOSIS SYSTEMIC LUPUS ERYTHEMATOSUS
- Systemic lupus erythematosus presenting with a reticular erythematous mucinosis-like condition.
Del Pozo J, Pena C, Almagro M, Yebra MT, Martinez W, Fonseca E.
Department of Dermatology and Pathology, Hospital Juan Canalejo, La Coruna, Spain.
Lupus. 2000;9(2):144-6. Abstract quote
We report the case of a woman with clinical and histopathological features of reticular erythematous mucinosis syndrome (REM) who six years later developed diagnostic criteria for systemic lupus erythematosus (SLE).
Two biopsies performed at the same area initially showed mucin dermal deposits and further development of characteristic dermo-epidermal changes of lupus erythematosus.
Our findings suggest that SLE may present with an REM-like condition.
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