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Brooke-Spiegler syndrome

Synonym(s) CYLD cutaneous syndrome
Prevalence -
Inheritance -
Age of onset -
ICD-10 -
  • C1857941
  • C536611
MedDRA -


Brooke-Spiegler syndrome (BSS) is a genodermatosis presenting with skin appendage tumors, including cylindromas, trichoepitheliomas, eccrine spiradenomas and major and minor salivary glands tumors. Familial cylindromatosis (FC) and multiple familial trichoepithelioma (MFT) (see these terms) represent the 2 ends within the spectrum of BSS. The disease follows an autosomal dominant inheritance scheme.

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