Abstract

IBIDS is a syndrome characterized by ichthyosis, brittle hair, impaired intelligence, decreased fertility and short stature. It is a subtype of trichothiodystrophy. We presented an 11-year-old girl whose clinical features fit into the IBIDS syndrome. She had congenital ichthyosis, brittle hair, intellectual impairment, possibly decreased fertility, short stature and the typical facial features of micrognathia and prominent ears. The hair showed a trichoschisis-like pattern at light microscopy and alternate banding at polarizing microscopy. Herein, we report a case and review available literatures.

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