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A Clinicopathologic Study of Four Patients

Evan R. Farmer, MD; George W. Hambrick, Jr, MD; Lawrence E. Shulman, MD, PhD

Arch Dermatol. 1982;118(1):9-13.

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 (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.

(Arch Dermatol 1982;118:9-13)

Author Affiliations
From the Department of Dermatology, The Johns Hopkins Medical Institutions, Baltimore. Dr Hambrick is now with the Division of Dermatology, The New York Hospital, New York, and Dr Shulman is now with the National Institutes of Health, Bethesda, Md.

Footnotes
Accepted for publication April 17, 1981.

Reprint requests to Department of Dermatology, The Johns Hopkins Medical Institutions, Baltimore, MD 21205 (Dr Farmer).

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