An 11-year-old Caucasian boy with good general health and normal development presented for evaluation of a congenital nevus on his leg. The physician noted that he had an unusual appearance, including bitemporal scars resembling forceps marks. The mother told us that he had had linear scar-like depressions on both temples since birth. Furthermore, the pregnancy and birth had been normal, and the delivery had not required the use of forceps or other instruments. The parents were healthy and the patient was normal, except for mild strabismus and hypermetropia. Examination revealed bilateral, hyperpigmented, depressed, sharply demarcated atrophic areas on the temples (Fig. 1). The other clinical manifestations were low frontal hairline, dense eyebrows, thick lips, long upper and lower eyelashes and a bulbous nose (Fig. 2).No other cranial or extracranial disorders were observed. Examination of his maternal grandmother revealed that she had similar scar-like lesions on both temples (Fig. 3). Histologic examination of a 3 mm punch biopsy specimen from a lesion on his right temple showed a thinned epidermis and dermis without skin appendages. Little elastic tissue was found in the dermis. Results of routine laboratory studies were within normal limits.
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