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Indian Journal of Dermatology, Venereology, Leprology, Vol. 71, No. 1, January-February, 2005, pp. 44-45 Letter To Editor Successful treatment of scleromyxedema with dexamethasone cyclophosphamide pulse therapy Kuldeep CM, Mittal AK, Gupta LK, Paliwal VKK, Sharma P, Garg A Departments of Dermatology, Venereology and Leprosy, RNT Medical College, Udaipur Code Number: dv05011 Sir, Scleromyxedema is an uncommon disorder of mucin deposition associated with paraproteinemia, especially IgG gammopathy.[1] Its pathogenesis is still uncertain, but there are various hypotheses, one of which suggests that some circulating factor, not the paraprotein, might be stimulating fibroblast activity.[2] Scleromyxedema shows little tendency for spontaneous remission. Its treatment is frequently disappointing and the evidence of therapeutic efficacy is largely anecdotal.[3] We would like to share our experience of using dexamethasone-cyclophosphamide pulse (DCP) therapy in a patient of scleromyxedema. We chose DCP therapy because of its successful use in auto-immune disorders like pemphigus, scleroderma and SLE.[4], [5] A 45-year-old male presented to us with gradually progressive asymptomatic hyperpigmentation and tightening of skin for three years. There was no history of Raynaud′s phenomenon. He was enjoying good general health otherwise. Personal and family history excluded silicosis, collagen vascular disease, thyroid disorder, neoplasm or drug reaction. Cutaneous examination revealed firm, closely set, lichenoid papules and plaques over the face, neck, buttocks and both extremities. The underlying skin showed generalized thickening and sclerosis. The skin of the forehead showed vertical furrows. Systemic examination was unremarkable. Routine hematological investigations revealed mild iron deficiency anemia and raised ESR (60 mm/1st hr). Serum biochemistry was normal. A chest X-ray and USG of the abdomen were normal. ANA test and thyroid function tests were normal. Serum electrophoresis did not reveal paraproteinemia. Histopathology of lesional skin revealed epidermal atrophy, and diffuse deposition of mucin in the papillary dermis that stained with Alcian blue. There was fibroblast proliferation, thickening and homogenization of collagen bundles with mild perivascular mononuclear infiltration. He was treated with dexamethasone-cyclophosphamide pulse therapy which consisted of 100 mg dexamethasone dissolved in 500 ml of 5% dextrose intravenous infusion given for 3 consecutive days along with cyclophosphamide 500 mg intravenously on day 2 every month. Therapy was well tolerated. Initial clinical improvement was noticed after the third pulse and a near total softening of skin was achieved after 24 DCP pulses. The mechanism through which DCP worked in our case is uncertain but it is likely that DCP either directly inhibits fibroblast proliferation or a circulating factor responsible for fibroblast proliferation. DCP seems to offer promise in this disorder that is difficult to treat. REFERENCES
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