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IMAGES IN CLINICAL PRACTICE
Year : 2016  |  Volume : 82  |  Issue : 6  |  Page : 738-739

Scleromyxedema


Institute of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College, Nanjing, Jiangsu, 210042, China

Date of Web Publication7-Oct-2016

Correspondence Address:
Dr. Jian-Fang Sun
Jiangwangmiao Street No. 12, Nanjing, Jiangsu, 210042
China
Dr. Wei Zhang
Jiangwangmiao Street No. 12, Nanjing, Jiangsu, 210042
China
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0378-6323.174420

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How to cite this article:
Yang Y, Chen H, Zhang W, Sun JF. Scleromyxedema. Indian J Dermatol Venereol Leprol 2016;82:738-9

How to cite this URL:
Yang Y, Chen H, Zhang W, Sun JF. Scleromyxedema. Indian J Dermatol Venereol Leprol [serial online] 2016 [cited 2019 Oct 15];82:738-9. Available from: http://www.ijdvl.com/text.asp?2016/82/6/738/174420


A 63-year-old male presented with a history of numerous papules on the neck, trunk and limbs for 2 years, partly in a linear arrangement, and progressive induration and thickening of the skin on the forehead and hands for 1 year [Figure 1]a,[Figure 1]b,[Figure 1]c. Biopsy of a papule on the left arm revealed Alcian blue-positive mucinous material between thick collagen bundles and proliferation of fibroblasts in the dermis. [Figure 1]d. Immunofixation electrophoresis revealed IgG monoclonal gammopathy with λ light chains. Thyroid gland function was normal.
Figure 1: Numerous firm papules and nodules distributed on the neck, trunk, and limbs (a), partly in a linear arrangement. Diffuse induration and thickening of the skin on the forehead (b) and hands (c). Positive alcian blue staining demonstrated mucin deposition (d)

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Scleromyxedema is a rare disease characterized by a generalized papular and sclerodermoid eruption, the histological triad of mucin deposition, fibroblast proliferation and fibrosis, monoclonal gammopathy, and absence of a thyroid disorder. Usually, treatment is unsatisfactory and systemic therapy may include melphalan, steroids, plasmapheresis, acitretin, immunoglobulin, thalidomide, methotrexate, cyclophosphamide, ciclosporin, and phototherapy. Recent reports recommended intravenous immunoglobulin (IVIG), which is relatively effective and safe but maintenance therapy is required to control disease.


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