Electron-beam therapy in scleredema adultorum with associated monoclonal hypergammaglobulinaemia

Abstract

We report a case of scleredema adultorum (Buschke's disease) associated with an IgA kappa monoclonal hypergammaglobulinaemia. A significant improvement in the skin was obtained with electron-beam therapy. Scleredema would appear to be linked to monoclonal hypergammaglobulinaemia, but the relationship between the skin disorder and the immunoglobulin abnormality remains to be elucidated.

Scleredema of Buschke Successfully Treated With Electron Beam Therapy

“…13 In that report, treatment of the patient's entire skin was accomplished using 20 Gy in 10 sessions. Our patient was treated with localized electron beam therapy, using the same dose of radiation.…”

Scleredema Diabeticorum Partially Treated with Low-Dose Methotrexate: a Report of Five Cases

“…Indeed, our first case was unaware of his diabetes and we did not have any information about the duration of his diabetes. Scleredema may be associated with systemic diseases such as multiple myeloma, hyperparathyroidism, malignant insulinoma, Gougerot-Sjögren syndrome or rheumatoid arthritis in the rare large series published in the literature (11,12). Although we examined all our five cases in the laboratuary assays, we did not observe any finding related with paraproteinemias or the other above-mentioned systemic diseases.…”

Therapy resistant idiopathic scleredema: an underlying pathology not always present

“…Nevertheless this pathology may also be idiopathic, as in our case. However, it should be kept in mind that monoclonal hypergammaglobulinemia may occur even years after the diagnosis of scleredema (3,5), and follow up should go on indefinitely. On the other hand, scleredema can be considered as a benign disease.…”