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Öğe Biotinidase Deficiency Accompanying Hair Changes and Periorificial Lesions: A Case Report(Galenos Yayincilik, 2011) Ayhan, Erhan; Kivrak, Abdullah; Aytekin, SemaBiotinidase 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.