Clinical Rheumatology

, Volume 2, Issue 3, pp 277–284 | Cite as

Papular mucinosis, destructive arthropathy median neuropathy, and sicca complex

  • R. A. Frayha
Case Report

Summary

A patient with papular mucinosis (scleromyxedema) developed an erosive seronegative rheumatoid-like arthropathy, carpal tunnel syndrome, sicca complex, and marked increase in TG(OKT8) suppressor/cytotoxic circulating T-cells akin to that reported in scleroderma. Sclerodactyly, acrolysis and stiff digits were striking but other features of scleroderma, i.e., Raynaud's and esophageal hypoperistalsis, were absent. The diagnosis of papular mucinosis, a pseudoscleroderma syndrome, should be considered in a patient with atypical arthritis, median neuropathy, myopathy, and/or sclerodactylyand a papular lichenoid dermatopathy. Skin mucin stain and the demonstration of the distinct serum paraprotein (PM-spike) are confirmatory. We stress the salient diagnostic clinical features of the leonine-like facies of papular mucinosis.

Key words

Papular Mucinosis Sjögren's Syndrome Scleromyxedema Paraproteinemia 

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Copyright information

© Springer-Verlag 1983

Authors and Affiliations

  • R. A. Frayha
    • 1
  1. 1.Department of Internal Medicine, Division of RheumatologyAmerican University of Beirut Medical CenterBeirutLebanon

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