Intravenous immunoglobulins control scleromyxoedema

Abstract

Background: Scleromyxoedema is a variant of papular mucinosis affecting the skin and internal organs. The different therapeutic approaches proposed for scleromyxoedema are still unsatisfactory. Intravenous immunoglobulin (IVIg) has been successfully employed in the treatment of connective tissue diseases and vasculitides.

Patients: The successful treatment of three cases of scleromyxoedema with IVIg is reported here.

Conclusions: The relatively low risk of the drug and the high effectiveness seen in three patients suggest that IVIg is a new treatment potentially useful in scleromyxoedema.

Figure 1

Patient 1 before the IVIg treatment (November 1998) showing characteristic skin lesion of scleromyxoedema. Papules can be seen on the ears and the forehead, and the face skin is thickened and hardened, reducing the ability to open the eyes and the mouth. Reproduced with permission of the patient.

Figure 2

Patient 1 after one month of IVIg treatment (March 1999). The characteristic skin lesions have disappeared. The skin of the face is softer and fewer papules are visible on the forehead. Reproduced with permission of the patient.