Papular mucinosis associated with myopathy.

Background, Papular mucinosis is an uncommon condition sometimes associated with myopathy as in our case. Case report. A 54-year-old woman presented several disseminated pruriginous papules that spread progressively. Histopathology of a skin biopsy showed mucine deposits and fibroblasts in the derma. Serum monoclonal lambda IgG w as evidenced. The patient complained of weakness of the proximal muscles an d dysphagia. Serum creatine phosphokinase was moderately elevated and the e lectromyography showed a typical myogenic pattern. Histopathology of a musc le biopsy disclosed a polymyositis aspect without mucine deposits. The pati ent was given prednisone. The skin lesions regressed totally with no modifi cation of the muscle deficit. Discussion. Myopathy associated with papulous mucinosis generally presents as a proximal deficit with dysphagia. Myalgia and amyotrophy are exceptiona l. Moderate serum myolysis is often evidenced. The electromyogram shows a m yogenic pattern. The pathophysiology of this condition remains unclear, but the association with papulous mucinosis does not appear to be fortuitous d ue to its frequency. No standard treatment has been established. General co rticosteroid therapy is generally effective and methotrexate, melphalan, is otretinoine and D-penicillamine have given good results in a few cases.