Treatment of scleromyxedema with melphalan

Abstract

Scleromyxedema is an uncommon cutaneous fibromucinous disease with a monoclonal protein, which has resisted a number of therapies. Eight cases followed up for as long as 12 years have provided an opportunity to observe the effects of melphalan treatment in this disease. The fibrohistiocytic and mucinous change of the skin in scleromyxedema and often the monoclonal protein can be controlled by low-dose chemotherapy. Although melphalan does not usually produce clinical toxic effects of importance, it is a myelotoxic drug and cytopenia is common; one patient died of acute myelomonocytic leukemia after ten years of successful therapy of the scleromyxedema, thus implying that long-term therapy may be dangerous by itself. These patients require close supervision. Leukocyte and platelet counts must be performed every three weeks, and the dosage of melphalan adjusted accordingly.

Scleromyxedema: A Case Report and Review of the Literature

“…Melphalan has produced some remarkable results in several severe cases, but its use has been restricted because of toxicity [12, 13, 14, 15, 16, 17, 18]. PUVA, Grenz ray, and electron beam therapy have also been used with mixed results [19].…”

Lichen myxoedematosus with associated cardiac abnormalities

“…1,2 There have been previous reports of poorly controlled hypertension. 3 Rudner et al reported grade 3 arteriolar sclerosis on fundoscopy. 4 Farmer et al 5 described autopsy findings in 12 individuals; seven had arteriosclerosis especially in the coronary arteries, and seven had neurological abnormalities, including cerebral atrophy, demyelination, cerebral thromboembolic disease and subdural haemorrhage.…”

“…Localized treatments to papular lesions have included intralesional injections of hyaluronidase and triamcinolone, with little success. 3,6 Surgical interventions using the carbon dioxide laser for dermabrasion have been used to good effect, giving a moderate cosmetic improvement, but this treatment does not prevent new lesions appearing. 7,8 Whole-body electron beam therapy was successful in reducing skin infiltration in patients with widespread skin involvement.…”

“…12±15 The antimetabolite melphalan has been used at doses averaging 1±2 mg daily (maximum 10 mg daily). This was given to eight patients over a period of up to 12 years by Harris et al 3 Their results ranged from no response to excellent results for both cyclical and continuous lowdose therapies. This treatment can be severely limited by leucopenia and thrombocytopenia.…”

Scleromyxedema, a Positive Effect with Thalidomide

“…Corticosteroids[7,8,9], PUVA [10], electron beam therapy [11], retinoids[ 12, 13], plasmapheresis[ 14], high-dose intravenous immunoglobulin[ 15], extracorporeal photochemotherapy[ 16], dermabrasion[ 17] and topical dimethyl sulfoxide [18] have all produced some symptomatic improvement; however, none of them have yielded long-term beneficial results. Several chemotherapeutic and modulatory agents such as mephalan[ 2, 18, 19], cyclosporine[ 20, 21], 2-chlorodeoxyadenosine[ 22], cyclophosphamide[ 23, 24], methotrexate[ 25, 26], chlorambucil[ 27] and a-interferon [28] have also been used in an attempt to interfere with the plasma cell dyscrasia. However, use of these agents is limited by toxicity and significant adverse side effects.…”