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Article
January 1987

Familial Subconjunctival Epithelial Cysts Associated With the Nevoid Basal Cell Carcinoma Syndrome

Author Affiliations

Department of Dermatology; Department of Pathology Emory University School of Medicine 1365 Clifton Rd NE Atlanta, GA 30322

Arch Dermatol. 1987;123(1):23-24. doi:10.1001/archderm.1987.01660250027006
Abstract

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

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