Scleredema

Orpha code: 352763OMIM code:

Definicja

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.

Disease data
Klasyfikacja

Disease

Synonimy
Buschke scleredema
Twardzina obrzękowa Buschkego
Kod ORPHA
352763
Kod OMIM
-
Kod ICD10
M34.8
Kod ICD11
EE7Y

No additional description.

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