%0 Journal Article
%T Atypical scleromyxedema improved by immunoglobulins intravenous
%V 10
%N 4
%P 352-354
%U http://www.odermatol.com/issue-in-html/2019-4-8-scleromyxedema/
%X Scleromyxedema is a rare condition that occurs in middle-aged people. The association with a monoclonal gammopathy is a main criterion for the diagnosis of typical forms. Its pathogenesis is poorly understood. Clinically characterized by a generalized papular eruption with firm papules of 2-3 mm in diameter, waxy in appearance, localised in the hands, forearms, head, neck, upper trunk and thighs. The most frequent extra-cutaneous manifestations include haematological, neurological, cardiac, digestive, rheumatologic and respiratory disorders. Histological findings show fibroblast proliferation, mucin deposition, and fibrosis. The low number of cases and the lack of randomized study make the therapeutic choice difficult. There is currently no formal consensus regarding its therapeutic management. Several therapeutic modalities have been reported such as steroids, retinoids, PUVA, interferon, plasmapheresis, chemotherapy, electrotherapy, thalidomide or immunoglobulins. The efficacy of immunoglobulins intravenous and their lower toxicity make them a treatment of choice for scleromyxedema. We report a case of isolated scleromyxedema with favorable evolution under immunoglobulins and Thalidomide.
%G en
%J Our Dermatology Online
%A Baybay, Hanane
%A Lamouaffaq, Amina
%A Bennani, Mounia
%D 2019-10-03