To the Editors.—
The nevoid basal cell carcinoma syndrome (NBCCS) is a multisystem hereditary syndrome involving both ectoderm and mesoderm. The mode of inheritance is autosomal dominant with variable penetrance, resulting in a marked variability of clinical presentation. Characteristically, patients present with multiple basal cell carcinomas of early onset, odontogenic keratocysts, palmar dyskeratosis, ectopic calcification, and skeletal abnormalities (bifid ribs, spina bifida, etc). Numerous clinical findings include ophthalmologic, endocrine, and central nervous system anomalies. Other cutaneous findings include epidermal cysts, milia, and lipomas. Epidermal cysts are occasionally seen in these patients, with an increased predilection for the palms. We present two patients in whom typical clinical findings of NBCCS are present, and who, in addition, have epithelial cysts of the palpebral conjunctival surfaces of the upper eyelids, an anatomic location not previously reported for these cysts.
Report of Cases.—Case 1.—
A 61-year-old man with NBCCS was evaluated at
Levine DJ, Robertson DB, Varma VA. Familial Subconjunctival Epithelial Cysts Associated With the Nevoid Basal Cell Carcinoma Syndrome. Arch Dermatol. 1987;123(1):23–24. doi:10.1001/archderm.1987.01660250027006
Customize your JAMA Network experience by selecting one or more topics from the list below.
Create a personal account or sign in to: