[Strip papular mucinosis associated with systemic sclerosis].

INTRODUCTION: Other than discreet infra-clinical mucinous deposits observed during many inflammatory dermatoses, clinically visible dermal mucinosis can sometimes be associated with collagenosis. It is usually lupus and very rarely scleroderma. In this case, no confusion with papular mucinosis must be made. CASE REPORT: A 51 year-old woman presented with infiltrated erythematous lesions in strips on the inner sides of the thighs and legs, associated with myalgia, arthralgia and puffy fingers. Mixed connective tissue disease was the initial diagnosis. The clinical picture was rapidly completed by sclerodactylia, telangiectasia, a Raynaud's syndrome and esophageal involvement leading to the diagnosis of CREST-type systemic scleroderma. The biopsy of the erythematous strip lesions revealed a dermal mucinosis. Treatment with hydroxychloroquine led to the regression of the mucinous lesions and the stabilization of the scleroderma, which, four years later, had not developed further. DISCUSSION: Dermal mucinosis can accompany lupus erythematosus, in rare cases dermatomyositis and, in exceptional cases, scleroderma. The clinical presentation varies with large infiltrated plaques, reticulated or papulo-nodular lesions. Conversely, strip lesions such as those observed in our patient have never been reported till now. The association of a localized dermal mucinosis and a scleroderma must not lead to the erroneous diagnosis of papular mucinosis of clearly differing prognosis. The occurrence of a mucinosis during collagenosis might be related to enhanced synthesis of mucin by the fibroblasts mediated by the inflammatory cytokines, increased in this context.