C205541Level 6
CYLD Cutaneous Syndrome
**Semantic type:** Disease or Syndrome
**Definition:** The development of multiple skin tumors including cylindromas, spiradenomas, trichoepitheliomas, and rarely, basal cell adenoma of the salivary gland. It usually manifests in the second or third decade. The tumors typically arise from the scalp and face but can also arise from the torso and sun-protected sites. It is caused by germline mutations of the tumor suppressor gene CYLD.
**Synonyms:** - CCS
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