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Giornale Italiano di Dermatologia e Venereologia 2000 April;135(2):217-21

Copyright © 2000 EDIZIONI MINERVA MEDICA

language: Italian

Reticular erythematous mucinosis syndrome (REM syndrome). Case report and therapeutical response to hydroxychloroquine sulfate

Brazzelli V., Chiesa M. G., Baldini F., Vassallo C., Ardigò M., Rosso R., Borroni G.

Università degli Studi - Pavia Policlinico S. Matteo IRCCS - Pavia Dipartimento di Patologia Umana ed Ereditaria Clinica Dermatologica * Istituto di Anatomia Patologica


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Reticular erythematous mucinosis (REM) syndrome is a rare, chronic disease characterized by asymptomatic, persistent reticular, slightly infiltrated erythematous plaques on chest and/or back with slight infiltration. Mild itching may be associated. At present, almost 100 patients with REM syndrome have been described. The case of a young woman with a pruritic, erythematous, reticular plaque on her back, worsened by sun exposure is described. Clinical and laboratory data were in the normal range or negative, the skin biopsy demonstrated the presence of mucin among collagen bundles and around blood vessels and adnexa; perivascular and periadnexial lymphocytic infiltration was present. Alcian blue pH 2.5 staining was strongly and diffusely positive. Direct immunofluorescence of the skin biopsy revealed the deposition of IgA, IgM, and fibrin around the papillary blood vessels, and complement at the dermoepidermal junction. Electron microscopy showed granular and filamentous material between the collagen fibres. All these features were consistent with a diagnosis of REM. Treatment with hydroxychloroquine sulfate 400 mg/day for a month and successively 200 mg/day, carotenoids and sun-screen creams resulted in a complete resolution of REM lesions. No recurrence of REM was seen at one year follow-up examination.

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