A Rare Case of Keratosis Follicularis Spinulosa Decalvans Affecting a Female Child

A 10-year-old girl with keratosis follicularis spinulosa decalvans (KFSD) presented with congenital alopecia and generalized rough skin. Examination revealed follicular papules, scarring alopecia, and xerosis. Histopathology confirmed KFSD, a rare condition often affecting males, linked to Xp22 mutations affecting cholesterol and lipid homeostasis. Despite treatment with topical clobetasol, no significant improvement was observed. KFSD's prognosis involves progressive follicular obliteration, and treatments, including corticosteroids and retinoids, have shown limited success. This case is notable for its rarity, especially in females, who typically exhibit milder symptoms.