Keratosis follicularis spinulosa decalvans (KFSD) is a rare, X-linked, hereditary disorder of keratinization, characterized by skin involvement and progressive scarring alopecia of scalp, eyebrows, and eyelashes. The diagnosis is helped by the particular clinical features, but pathology is mandatory. Case Presentation: We described a case of a female patient referred to the outpatient's hair consultation of our department, in which we performed trichoscopy as a very useful tool for the diagnosis, followed by pathology that confirmed KFSD. Conclusion: In our article, we underlined the importance of trichoscopy for the diagnosis of this hair disease, with also a review of the literature on diagnosis and treatment.
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