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


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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  1. 1.
    Ghanda, J.J. Scleromyxedema. Cutis, 1979, 24, 549–552.PubMedGoogle Scholar
  2. 2.
    Frayha, R.A., Masri, A.F., Zaynoun, Sh., Kurban, A., Nsouli, Gh. The rheumatic features of papular mucinosis: A pseudoscleroderma syndrome (abs). Arthritis Rheum, 1982, 25 (4), S 127.Google Scholar
  3. 3.
    Hill, T., Crawford, J., Rogers, C. Successful management of lichen myxedematosus. Arch Dermatol, 1976, 112, 67–69.CrossRefPubMedGoogle Scholar
  4. 4.
    Jakubovic, H., Salama, S., Rosenthal, D. Multiple cutaneous focal mucinosis with hypothyroidism. Ann Intern Med, 1982, 96, 56–58.PubMedGoogle Scholar
  5. 5.
    Danby, F., Danby, C., Pruzanski, W. Papular mucinosis with IgG (K)M component. Can Med Assoc J, 1976, 114, 920–922.PubMedGoogle Scholar
  6. 6.
    Lawrence, D., Tye, M., Liss, M. Immunochemical analysis of the basic immunoglobulin in papular mucinosis. Immunochem, 1972, 9, 41–49.CrossRefGoogle Scholar
  7. 7.
    Wright, R., Franco, R., Denton, M., Blaney, D. Scleromyxedema. Arch Dermatol, 1976, 112, 63–66.CrossRefPubMedGoogle Scholar
  8. 8.
    Wells, J., Fundenberg, H., Epstein, W. Idiotypic monoclonal immunoglobulins. J Immunol, 1972, 108, 997–983.Google Scholar
  9. 9.
    Crossland, P.M. Fibromucinodosus (lichen myxedematosus). Arch Dermatol Syphilig, 1953, 67, 122–123.Google Scholar
  10. 10.
    Montgomery, H., Underwood, L. Lichen myxedematosus. J Invest Dermatol, 1953, 20, 213–236.PubMedGoogle Scholar
  11. 11.
    McAdam, L., Pearson, C., Pitts, W., Sadoff, L., Verity, M.A. Papular mucinosis with myopathy, arthritis, and eosinophilia. Arthritis Rheum, 1977, 20, 989–996.PubMedGoogle Scholar
  12. 12.
    Harris, R., Perry, H.O., Kyle, R., Winkelmann, R. Treatment of scleromy oedema with melphalan. Arch Dermatol, 1979, 115, 295–299.CrossRefPubMedGoogle Scholar
  13. 13.
    MacCarthy, J., Osserman, E., Lombardo, P., Taktasuki, K. An abnormal serum globulin in lichen myxedematosus. Arch Dermatol, 1964, 89, 446–450.PubMedCrossRefGoogle Scholar
  14. 14.
    Byrd, W.E. Personal communication.Google Scholar
  15. 15.
    Perry, H.O., Montgomery, H., Stickney, J. Further observations on lichen myxedematosus. Ann Intern Med, 1960, 53, 955–969.PubMedGoogle Scholar
  16. 16.
    Johnson, B.L., Horowitz, I.R., Charles, C.R. Dermatomyositis and lichen myxedematosus. Dermatologica 1973, 147, 109–122.PubMedCrossRefGoogle Scholar
  17. 17.
    Wigley, J., Rees, D., Symmers, W. Lichen myxedematosus. Br J Dermatol 1957, 69, 408–410.PubMedGoogle Scholar
  18. 18.
    Hardie, R., Hunter, J., Urbaniak, S., Habeshaw, J. Spontaneous resolution of lichen myxedematosus. Br J Dermatol 1979, 100, 727–730.PubMedGoogle Scholar
  19. 19.
    Nagy, E., Szodoray, L., Pongracz, E. Gleichzeitiges Vorkommen Von Hashimotoscher Struma, Dermatomyositis and Skleromyxoedem. Z Hautkr, 1962, 33, 26–29.Google Scholar
  20. 20.
    Verity, M.A., Toop, J., McAdam, L., Pearson, C. Scleromyxedema myopathy. Histochemical and electron microscopic observations. Am J Clin Path, 1978, 69, 446–451.PubMedGoogle Scholar
  21. 21.
    Cipoletti, J., Buckingham, R., Barnes, E.L., Peel, R., Mahmood, Kh., Cignetti, F., Pierce, J., Rabin, B., Rodnan, G. Sjögren's syndrome in progressive systemic sclerosis. Ann Intern Med, 1977, 87, 535–541.PubMedGoogle Scholar
  22. 22.
    Gupta, S., Malaviya, A., Rajagopalan, P., Good, R. Subpopulations of human T lyphocytes. IX imbalance of T-cell subpopulations in patients with progressive systemic sclerosis. Clin Exp Immunol, 1979, 38, 342–347.PubMedGoogle Scholar
  23. 23.
    Krawitt, E., Holdstock, G., Bland, J., Ghastenay, B., Albertini, R. Suppressor cell activity in progressive systemic sclerosis. J Rheumatol, 1982, 9, 263–367.PubMedGoogle Scholar
  24. 24.
    Proppe, A., Becker, V., Hardmeier, T. Sckleromyxodem Ardnt-Gottron and plasmocytom. Hautartz 1969, 20, 53–59.Google Scholar
  25. 25.
    Harper, R.A., Rispler, J. Lichen myxedematosus serum stimulates human skin fibroblast proliferation. Science 1978, 199, 545–547.PubMedGoogle Scholar
  26. 26.
    Shapiro, C.M., Fretzin, D., Norris, S. Papular mucinosis. JAMA, 1970, 214, 2052–2054.CrossRefPubMedGoogle Scholar
  27. 27.
    Kallen, P.S., Nies, K.M., Louie, J.S. Scleromyxedema — a sclerodermoid disorder (letter). J Rheumatol, 1981, 8, 526–528.PubMedGoogle Scholar

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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