Focal dermal hypoplasia. A nine-year follow-up study

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.