Scleromyxedema (lichen myxedematosus) associated with dermatomyositis

A 41-year-old white man is described with papules of the lower and upper ba ck, the neck and the upper chest, a marked deposition of mucin in the upper reticular dermis, and an Ige A monoclonal gammopathy strongly evocative of scleromyxedema (lichen myxedematosus). Additionally, he developed intense myalgia, muscle weakness and rhabdomyolysis, which were associated with hel iotrope erythema, photosensitivity and an erythematous rash of the dorsum o f the hands with Gottron's papules. Muscle biopsy revealed an inflammatory myositis, and dermatomyositis was diagnosed. The association of dermatomyos itis and secondary mucinosis, or muscle involvement in primary papular muci nosis are not rare. However, the association between scleromyxedema and der matomyositis has only exceptionally been reported.