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September 1981

Monoclonal Gammopathy in Scleredema: Observations in Three Cases

Author Affiliations

From the Departments of Dermatology, University of Münster (Drs Kövary, Vakilzadeh, and Macher); Städtische Krankenanstalten Reinkenheide, Bremerhaven (Dr Zaun); and the University of Düsseldorf (Drs Merk and Goerz), West Germany. Dr Kövary is now with Universitäts-Hautklinik, Münster, West Germany.

Arch Dermatol. 1981;117(9):536-539. doi:10.1001/archderm.1981.01650090018016

• A monoclonal gammopathy was observed in three patients with long-term and widespread scleredema (Buschke's disease). There was no evidence of multiple myeloma in any patient. Deposition of monoclonal immunoglobulins in the skin was not detected by direct immunofluorescence microscopy. In contrast to scleromyxedema (lichen myxedematosus), from which scleredema can be distinguished clinically and histologically, the monoclonal immunoglobulins in two cases were of IgG - K and IgG3-K type. Only one of the three patients had IgG1-λ paraproteinemia, which is frequently seen in scleromyxedema. Our findings suggest that diffuse scleredema may be characterized by paraproteinemia but that the possible role of monoclonal immunoglobulins in the pathogenesis of this disease has yet to be resolved.

(Arch Dermatol 1981;117:536-539)

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