Description of the disease * EnglishPolish Pobierz sekcję do PDF Definicja A rare lichen myxedematosus characterized by a progressive, generalized, papular, sclerodermoid cutaneous eruption usually occurring in association with monoclonal gammopathy, but in the absence of thyroid disease. Histological hallmark is the triad of dermal mucin deposition, fibroblast proliferation, and fibrosis. Patients present with relatively sudden onset of numerous closely spaced, waxy, firm papules and plaques predominantly involving the head, neck, trunk, and dorsal aspects of the extremities, on the background of thickened, edematous, erythematous skin with sclerodermoid appearance. Systemic involvement with cardiovascular, gastrointestinal, pulmonary, musculoskeletal, renal, or nervous system complications is common. Disease data Klasyfikacja Disease Synonimy Arndt-Gottron disease Choroba Arndta i Gottrona Uogólniona wysypka grudkowa liszajowata Generalized lichenoid papular eruption Generalized papular and sclerodermoid lichen myxedematosus Kod ORPHA 167635 Kod OMIM - Kod ICD10 L98.5 Kod ICD11 EB90.11 *Soruce Extended description of the disease Pobierz sekcję do PDF No additional description. Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl