기저세포모반증후군과 관련 없이 발생한 피부각질낭 1예
Sporadic Cutaneous Keratocyst without Nevoid Basal Cell Carcinoma Syndrome - Report of 1 Case -
이혜원, 박지영, 강선희, 최미선,
소속 상세정보
이혜원 ( Lee Hye-Won )
계명대학교 의과대학 병리학교실
박지영 ( Park Ji-Young )
계명대학교 의과대학 병리학교실
강선희 ( Kang Sun-Hee )
계명대학교 의과대학 외과학교실
최미선 ( Choe Mi-Sun )
계명대학교 의과대학 병리학교실
KMID : 0357920110450030322
Abstract
A cutaneous keratocyst is very rare and is ordinarily associated with nevoid basal cell carcinoma syndrome (NBCCS), also known as Gorlin syndrome. NBCCS is a rare autosomal-dominant disorder that results from the mutation in the patched homologue 1 (PTCH1) gene located on chromosome 9q22.3, with high penetrance and variable expressivity. NBCCS demonstrates multisystem manifestations such as multiple basal cell carcinomas in early age, jaw cysts and pits of the hands and feet. Cutaneous keratocysts are characteristically lined by festooned keratinized squamous epithelium with parakeratosis. The cystic wall contains neither granular cell layer nor skin appendages. To the best of our knowledge, only two cases of cutaneous keratocysts not associated with NBCCS have been reported to date. We report one another case of a histologically con-firmed cutaneous keratocyst in a 50-year-old female without a family history and clinical features of NBCCS.
키워드
Keratocysts; Nevoid basal cell carcinoma syndrome; Skin
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