Brooke-Spiegler syndrome (BSS) is an inherited autosomal dominant disease characterized
by the development of multiple adnexal cutaneous neoplasms most commonly …

Context.—Skin neoplasms include the most common malignancies affecting humans. Many
show an ultraviolet (UV)–induced pathogenesis and often affect the head and neck region …

Background & aims Monogenic forms of inflammatory bowel disease (IBD) illustrate the
essential roles of individual genes in pathways and networks safeguarding immune …

CYLD cutaneous syndrome (CCS) typically manifests in the second or third decade with the
appearance of multiple skin tumors including cylindromas, spiradenomas …

Genetic alterations of CYLD lysine 63 deubiquitinase (CYLD), a tumor-suppressor gene
encoding a deubiquitinase (DUB) enzyme, are associated with the formation of tumors in …

Abstract Brooke-Spiegler syndrome (BSS; OMIM 605041) is an autosomal dominant
condition characterized by skin appendageal neoplasms including cylindromas …

Cylindroma is a rare skin tumour that is inherited in several skin-tumour syndromes caused
by germline mutations in the tumour suppressor gene, CYLD. In this Review, we provide …

Spiradenocarcinoma is a rare skin adnexal neoplasm with potential for aggressive behavior,
classified histologically into low-and high-grade tumors. Morphologically, low-grade tumors …

Cutaneous cylindroma is an adnexal tumour with apocrine differentiation. A predisposition to
multiple cylindromas is seen in patients with Brooke–Spiegler syndrome, who carry germline …

Germline CYLD mutation is associated with the development of a rare inheritable syndrome,
called the CYLD cutaneous syndrome. Patients with this syndrome are distinctly presented …