Eyelid basal-cell carcinomas are the most common ocular finding of this syndrome. These malignancies may be multiple and may occur on the neck, chest, back, arms and elsewhere on the face. Those on the eyelids generally have their onset in the postpubertal period, usually by age 35 years, and are often multiple. Their indolent nature can result in considerably delay in diagnosis, however, and local recurrences are common. Deformities of the skull often result in the appearance of hypertelorism and proptosis. Epidermal cysts are found in one-fourth of patients, especially on the palms, but may occur in the tarsal conjunctiva as well. Intratarsal keratinous eyelid cysts occur in 40% of patients. Less common reported ocular findings are colobomas, glaucoma, nystagmus, strabismus, and cataracts but these may simply be associations.
