What lies beneath the skin.

نویسندگان

  • Mrisa Sahai
  • Alyce M Oliver
چکیده

To cite: Sahai M, Oliver AM. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/bcr-2013008584 DESCRIPTION A 46-year-old man with a history of hypertension presented with a 6-month history of progressive, diffuse skin thickening. He denied any exposure to new drugs, toxins or contrast material, had no history of Raynaud’s disease or endocrine disorder. Physical examination revealed diffuse skin tightness, limited range of motion of joints and sparse hair with leonine facies (figures 1 and 2). A skin biopsy revealed fibroblast proliferation (figure 3) and special staining with Alcian Blue (figure 4) revealed an increased mucin between and among collagen bundles resulting in the diagnosis of scleromyxedema. Further work-up revealed a bone marrow biopsy consistent with a plasma cell dyscrasia. Patient was subsequently treated with dexamethasone, bortezomib and thalidomide resulting in stabilisation of skin fibrosis and resolution of plasma cell dyscrasia. Scleromyxedema is a rare connective tissue disorder that has been associated with paraproteinemias. It is characterised by cutaneous eruption of small, waxy, firm papules commonly occurring over

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عنوان ژورنال:
  • BMJ case reports

دوره 2013  شماره 

صفحات  -

تاریخ انتشار 2013