Sporadic Cutaneous Keratocyst without Nevoid Basal Cell Carcinoma Syndrome: Report of 1 Case
Korean Journal of Pathology
;
: 322-325, 2011.
Artículo
en Coreano
| WPRIM
| ID: wpr-47955
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 confirmed cutaneous keratocyst in a 50-year-old female without a family history and clinical features of NBCCS.
Texto completo:
Disponible
Índice:
WPRIM (Pacífico Occidental)
Asunto principal:
Paraqueratosis
/
Piel
/
Carcinoma Basocelular
/
Síndrome del Nevo Basocelular
/
Quistes Maxilomandibulares
/
Quistes Odontogénicos
/
Penetrancia
/
Epitelio
/
Pie
/
Mano
Límite:
Femenino
/
Humanos
Idioma:
Coreano
Revista:
Korean Journal of Pathology
Año:
2011
Tipo del documento:
Artículo
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