Familial cylindromatosis (FC) is a rare, autosomal dominant disorder characterized by the development of multiple benign tumors originating from the skin appendages. It is linked to CYLD gene, whose loss of function impairs epidermal differentiation.
Category
Neoplasm
Brite
Human diseases in ICD-11 classification [BR:br08403]
02 Neoplasms
Benign neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues
Benign neoplasms except of mesenchymal origin
Benign cutaneous neoplasms
2F22 Benign neoplasms of epidermal appendages
H00828 Familial cylindromatosis
Pathway-based classification of diseases [BR:br08402]
Cellular processes
nt06527 Necroptosis
H00828 Familial cylindromatosis
