Scleromyxedema

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Synonyms: Arndt-Gottron disease | Generalized lichenoid papular eruption | Generalized papular and sclerodermoid lichen myxedematosus

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.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview. Reference: Access aggregated data from Orphanet at Orphadata. Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version May 2024

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