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A 4-YEAR-OLD girl had poor hair growth since birth. At birth her hair was coarse and kinky, and it fell out when she was 8 weeks old. Within months, some hair grew on her central scalp; however, she remained bald in the occipital and temporal areas for more than 1 year. By 2 years of age she had brittle hair of variable lengths throughout her scalp. Hairs were shortest in areas of greatest friction, and her mother noted that they broke easily even with gentle handling. The child required few haircuts.

With the exception of recurrent otitis media, she was otherwise healthy. There was no known family history of problems with the hair or skin. The child's growth and development were normal. Hearing evaluations revealed no abnormal findings.

On physical examination, she seemed healthy and had blonde hair that shimmered in reflected light. Her hair was coarse, fragile, and ranged in length from approximately 1 to 7 cm. Broken hairs were most prominent in the occipital region (Figure 1). Her eyebrows were sparse. Her nails, teeth, eyes, and skin were normal. Light microscopic examination of cut hairs from the child's scalp was performed (Figure 2 and Figure 3).