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Monoclonal Gammopathy in Scleredema:  Observations in Three Cases

Peter Michael Kövary, MD; Fereydoun Vakilzadeh, MD; Egon Macher, MD; Hansotto Zaun, MD; Hans Merk, MD; Günter Goerz, MD
Arch Dermatol. 1981;117(9):536-539. doi:10.1001/archderm.1981.01650090018016.
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• 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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