SCLEREDEMA ASSOCIATED WITH A PARAPROTEIN

There are three types of scleredema:

Clinical

Diffuse induration of the skin of the face, neck, shoulders, trunk and extremities may develop in a patient with a paraprotein and or with a malignant insulinoma. The paraprotein is usually of the IgA or IgG type. It often progresses to myeloma. Rarely, the disease may be so severe as to limit facial mobility, the ability to eat, and even to breath. Such cases may be fatal.

Workup

Patients should be evaluated for myeloma.

Treatment

A systemic corticosteroid is usually tried initially. In those cases associated with myeloma, the skin changes may improve with treatment of the myeloma. Electron-beam therapy resulted in "spectacular" clinical improvement in one patient. Extracorporeal photopheresis was successful in another case. Methotrexate improved the induration in another [Acta Dermatovenerol Croat. 2011;19(3):191-4].


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