Papular mucinosis. A clinicopathologic study of four patients

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Abstract
Four patients with papular mucinosis were studied. Each patient had clinically characteristic cutaneous lesions, increased dermal deposition of mucin, and a serum monoclonal IgG paraprotein. One patient had sclerodermoid features consistent with the scleromyxedema variant of papular mucinosis. Associated findings in the patients included pachydermoperiostosis (1 case), adenocarcinoma of the stomach (1-case), carcinoma of the pancreas (1 case), bizarre neurologic symptoms (1 case), and emphysema (2 cases). Autopsies were performed in 2 cases and no increased mucin deposition was observed in internal organs. Immunofluorescence microscopic study of involved skin showed no immunoreactant deposition in the 3 patients studied. Observations support the hypothesis that papular mucinosis is a disorder of skin fibroblasts without internal organ involvement.