Scleromyxedema, a Positive Effect with Thalidomide

Abstract

Scleromyxedema is a rare dermatological disorder marked by widespread symmetric 2- to 3-mm, firm, waxy, closely spaced papules involving the hands, forearms, face, neck, upper trunk and thighs. The most common extracutaneous manifestation of scleromyxedema is a benign plasma cell dyscrasia. Treatment of scleromyxedema is limited by the lack of long-term results, toxicity and significant adverse side effects. We report a severe case of scleromyxedema who had marked improvement when treated with thalidomide.

Rapid response of plasmacytomas to lenalidomide plus low‐dose dexamethasone therapy in a patient with relapsed multiple myeloma

“…The majority of patients with scleromyxedema have a concomitant monoclonal gammopathy of unknown significance. The serum of these patients has been shown to induce fibroblast proliferation in vitro, as well as increase fibroblast production of hyaluronic acid and prostaglandin E; however, the purified immunoglobulin alone does not induce these effects in vitro [14,15]. The level of paraproteinemia does not correlate with skin or systemic findings [14,15].…”

“…The serum of these patients has been shown to induce fibroblast proliferation in vitro, as well as increase fibroblast production of hyaluronic acid and prostaglandin E; however, the purified immunoglobulin alone does not induce these effects in vitro [14,15]. The level of paraproteinemia does not correlate with skin or systemic findings [14,15]. It has been suggested that the paraproteinemia may be a response to an unknown inciting factor and not the cause of disease manifestations.…”

“…Diagnosis requires the combination of the characteristic generalized papular sclerodermoid eruption, histological findings of increased fibroblasts and mucin, presence of a monoclonal gammopathy, and the absence of thyroid disease [16,17]. correspondence Scleromyxedema is associated with a myriad of systemic manifestations including dysphagia, dysarthria, dyspnea, weakness, peripheral neuropathy, carpal tunnel syndrome, arthritis, myalgias, myositis, myocardial infarction, restrictive lung disease, cerebral vascular accidents, memory loss, gait problems, fevers, encephalopathy, seizures, and coma [1,9,10,14,15]. Esophageal dysmotility is the most common extracutaneous manifestation [14].…”

“…A number of therapies have been reported to be varyingly successful in case reports and case series including high dose systemic corticosteroids, psoralen with ultraviolet A (UVA), electron beam radiation, retinoids, plasmapharesis, IVIG, extracorporeal photochemotherapy, dermabrasion, mephalan, cyclosporine, cyclophosphamide, methotrexate, chlorambucil, a-interferon, autologous stem cell transplant (ASCT) after conditioning with melphalan and thalidomide, ASCT after conditioning with carmustine, etoposide, cytarabine, and melphalan (BEAM), and thalidomide [14,15,18]. Treatments with thalidomide and IVIG, sometimes in combination, have been reported most frequently [15,19]. Patients with dermato-neuro syndrome have been reported to respond well to systemic steroids, plasmapheresis and IVIG [2][3][4][5][6][7][8][9][10][11][12].…”

Scleroderma-like Fibrosing Disorders

“…Case reports cite variable improvement with other immunosuppressants including cyclophosphamide and cyclosporine (65)(66)(67)(68). There are multiple cases describing some benefit using thalidomide, although there remains a legitimate concern for the development of disabling peripheral neuropathy (68)(69)(70)(71)(72). More recent data note benefit of autologous stem cell transplantation in recalcitrant cases (53,(73)(74)(75).…”