Woolly Hair Syndrome: Clinical and Microscopic Study

The study reported on three cases of woolly hair syndrome within the same family, identifying it as a dominant autosomal trait. The affected individuals exhibited fine, soft, frizzy hair and generalized hypotrichosis. In one case, atrophic follicular keratosis developed during puberty. Laboratory tests showed normal serum and urinary copper and zinc levels. Microscopic examination of the hair revealed several abnormalities, including flat, oval, or irregular hair shafts, longitudinal and transverse grooves, irregular axial torsions, and damage or absence of cuticle cells in the distal hair, leading to early hair fractures such as trichorrhexis nodosa, trichoschisis, and trichoptilosis.