Immunochemical Analysis of the Monoclonal Paraprotein in Scleromyxedema

Abstract

The monoclonal paraprotein from a typical case of scleromyxedema was isolated and characterized. The isolated paraprotein was of the IgG-lambda class, with a molecular weight of approximately 110,000 daltons compared with 160,000 daltons for normal IgG. Immunochemical studies indicated that the paraprotein was an incomplete IgG molecule which was missing a significant antigenic portion of the Fd fragment.

Discoid lupus erythematosus and monoclonal gammopathy

“…A monoclonal gammopathy occurs in about io"o of patients with pyoderma gangrenosum, and IgA is the predominant class (Powell et al, 1983), which is also the type associated with subcorneal pustular dermatosis (Cream, Grimes & Roberts, 1977;Wallach et al, 1982) and erythema elevatum diutinum (Kovary et al, 1981). In lichen myxoedematosus, the abnormal protein is predominantly IgG / (Kitamura et al, 1978). An IgG monoclonal protein has also been found in patients with scleroedema of Buschke (Kovary et al, 1981).…”

“…Monoclonal gammopathy has been associated with other cutaneous diseases. In some instances (for example, necrobiotic xanthogranuloma) the paraproteinaemia is present in most cases, almost as a marker of the disease (Kossard & Winkelmann, 1980), while in others a particular class of monoclonal protein is seen. A monoclonal gammopathy occurs in about io"o of patients with pyoderma gangrenosum, and IgA is the predominant class (Powell et al, 1983), which is also the type associated with subcorneal pustular dermatosis (Cream, Grimes & Roberts, 1977;Wallach et al, 1982) and erythema elevatum diutinum (Kovary et al, 1981).…”

Nephrogenic systemic fibrosis: Clinicopathological definition and workup recommendations

“…19 When indicated, a bone-marrow aspirate may be obtained. 20 Eosinophilic fasciitis, which is often accompanied by peripheral eosinophilia, 21 may resemble NSF.…”

What lies beneath the skin

“…Histologically, there is a cutaneous deposition of mucin in the superficial dermis and fibroblast proliferation. The presence of a monoclonal protein, often IgG-λ, supports the diagnosis of scleromyxedema 1. Treatment with thalidomide, melphalan or high-dose intravenous immunoglobulin may reduce the paraprotein 2…”