ABSTRACT

Biotinidase deficiency is impairment of biotin metabolism characterized by various dermatological,

ophthalmic and neurological symptoms. Autosomal recessive trait is a disorder. Skin findings such as alopecia, periorificial dermatitis and seborrhoeic dermatitis lesions are seen.

Clinical signs improved dramatically with biotine treatment. We presented a 6-year-old male patient with periorificial lesions, alopecia and microscopic hair shaft defects. (Turk J Dermatol 2011; 5: 79-81)