Papular mucinosis. A clinicopathologic study of four patients

Abstract

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