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Synonyms: Buschke scleredema

A rare acquired skin disease characterized by excessive mucin deposition and thickened collagen bundles in the dermis resulting in woody non-pitting induration of the skin of the neck spreading to the shoulders and upper trunk but sparing hands and feet. According to the association with preceding or underlying conditions three types can be distinguished: type 1 usually follows a febrile infection type 2 is associated with paraproteinemia and type 3 occurs in patients with diabetes mellitus. Especially in types 2 and 3 extracutaneous involvement may be present. Other potentially associated conditions include a variety of endocrinopathies systemic diseases and neoplasms.

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 Data version July 2024

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