Scleromyxedema: Successful Treatment with Thalidomide in Two Patients

Abstract

Background: Scleromyxedema is an unusual skin disease characterized by mucinous infiltration of the skin. There is no satisfactory treatment for this disorder. Case Report: We report 2 cases of scleromyxedema, both associated with an IgG-λ paraprotein. Treatment with thalidomide was started and great improvement of the lesion was noted as shown by a quality of life questionnaire similar to those used in rheumatoid arthritis. TNF-α value was elevated in both cases during this treatment. Discussion: We hypothesize that the efficiency of thalidomide could be mediated by its costimulatory effect on T cytotoxic lymphocytes.

Thalidomide affects the skeletal system of ovariectomized rats

“…Thalidomide has been approved for the treatment of multiple myeloma and erythema nodosum leprosum. Clinical trials are currently being conducted for its use in treating inflammatory diseases, various neoplastic diseases, complications of human immunodeficiency virus infection, chronic graft-versus-host disease and numerous dermatological conditions, among others [1,13,19,23,25,27,29,30,37,44].…”

Scleromyxedema with Subcutaneous Nodules: Successful Treatment with Thalidomide and Intravenous Immunoglobulin

“…Based on previous reports [10, 24, 25], we decided to treat our patient initially with thalidomide. After 3 months, we observed only a moderate improvement of the cutaneous lesions.…”

Vincristine, Idarubicin, Dexamethasone and Thalidomide in Scleromyxoedema

“…The rationale for using thalidomide as maintenance therapy in patients with scleromyxoedema was its recognized role in the treatment of refractory multiple myeloma, in which marked and durable responses counteracting plasr)ia cell dyscrasia were obtained with reduction of paraprotein levels (18)(19)(20)(21)(22). Moreover, thalidomide has been proposed to be an anti-fibrotic agent through its immuiioregulatory effect on pro-inflammatory and pro-fiblrotic cytokines in myelodysplastic syndrome with bone-marrow fibrosis and the decrease in gammaglobulin levels in patients with systemic lupus erythematosus ( 19)L Several case studies of scleromyxoedema indicate a benefit from single long-term thalidomide at a dosage of 150^00 mg/dayj (18)(19)(20)(21)(22) if patients undergo strictly controlled contraception as well as baseline and biannual nerve studitis (23).…”

“…Moreover, thalidomide has been proposed to be an anti-fibrotic agent through its immuiioregulatory effect on pro-inflammatory and pro-fiblrotic cytokines in myelodysplastic syndrome with bone-marrow fibrosis and the decrease in gammaglobulin levels in patients with systemic lupus erythematosus ( 19)L Several case studies of scleromyxoedema indicate a benefit from single long-term thalidomide at a dosage of 150^00 mg/dayj (18)(19)(20)(21)(22) if patients undergo strictly controlled contraception as well as baseline and biannual nerve studitis (23). However, the development of neuropathy as a significant adverse event remains a major limitation tO:an acceptable risk-to-benefit ratio, as documented also in our patient.…”