Spinular follicular keratosis of Siemens associated with wooly hair in two sisters: Trichoscopic description and anatomopathological correlation

Abstract

Spinular follicular keratosis (SFK) is a rare X-linked inherited disorder of keratinization characterized by diffuse follicular hyperkeratosis and progressive scarring alopecia of the scalp, eyebrows, and eyelashes. Trichoscopy is a non-invasive dermatological tool that has improved the differential diagnosis of hair pathologies. In fact, dermoscopic models have been developed for several entities of scalp diseases, allowing early diagnostic confirmation and rapid action without the need for histopathology, as well as good follow-up for patients and signs of pathological activity. To date, the specific trichoscopic signs of KFSD have not been developed. Herein, we report the case of KFSD of Siemens in two sisters associated with wooly hair. We employed trichoscopy as a highly useful tool for diagnosis, then performed an anatomopathological study to confirm the diagnosis and explain the signs found on trichoscopy.