WOOLLY HAIR SPORADICO

Woolly hair is a pilar dysplasia characterized by curly hair which is difficult to brush, occurring at birth or in the first months of life. It can be either genetically determined or nonhereditary. It can occur localized or diffuse. In the latter case woolly hair is classified in two main forms, namely hereditary woolly hair transmitted in autosomal dominant fashion, and familial woolly hair in which an autosomal recessive inheritance is hypothesized. The authors report a case of woolly hair in a 14-month-old female who was referred because of febrile seizures. The hair disorder, present from the first month of life, was neither familial nor associated with other cutaneous or systemic symptoms. A hypotrichosis of eyelashes and eyebrows was not present. Scanning electron microscopic analysis showed flattened hair, rounded or oval cross-sections, twists at irregular intervals, and mild cuticle damage. Woolly hair does not present specific ultrastructural abnormalities. It can be rarely observed in association with important extra-cutaneous anomalies. The present case, in absence of familial cases, appears sporadic probably due to a new mutation.