Focal Dermal Hypoplasia

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Abstract
The only patient known to have unilateral anophthalmia as part of the syndrome of focal dermal hypoplasia (Goltz syndrome) was reexamined dermatologically after a lapse of nearly nine years. In addition to the more severe anetoderma, depigmentation, and nail dystrophy, the patient had developed perioral wart-like lesions. Other new findings included malformed teeth with hypoplastic enamel, mental retardation, and possible urinary abnormality. A review of the literature disclosed two more cases of apparent focal dermal hypoplasia; these were previously reported as other entities. Contrary to earlier thought, the condition may be found in males.

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