Familial keratosis follicularis spinulosa decalvans associated with woolly hair

Abstract

Keratosis follicularis spinulosa decalvans (KFSD) is a rare inherited disorder of keratinization clinically characterized by diffuse follicular hyperkeratosis, progressive scarring alopecia of scalp, eyebrows and eyelashes, corneal dystrophy and photophobia. Woolly hair is a hereditary condition, transmitted as an autosomal dominant or recessive trait, usually seen in Caucasians at birth or shortly after, in which there are curly, thick, often heavily pigmented hairs. We report two cases, a son and his mother, in whom KFSD occurred in association with woolly hair. In addition, various dental anomalies, including agenesis, inclusions and teeth malformations, were present in the child. Interestingly, both patients simultaneously developed an inflammatory tinea capitis caused by Microsporum canis. To our knowledge, the association of KFSD with woolly hair has not been described. The dental anomalies found in the child are intriguing, as they have never been reported previously in patients with KFSD. Finally, the concomitant onset of inflammatory tinea capitis in both patients may be explained by the enhanced susceptibility to fungal infection in keratinizing disorders. © 2007 The International Society of Dermatology.