Keratosis follicularis spinulosa decalvans (KFSD) is a rare genodermatoses characterized by hyperkeratotic follicular papules on the scalp, with progressive cicatricial alopecia of the scalp, eyelashes, and eyebrows. Ocular involvement with photophobia and keratitis, and dental anomalies may also be seen. Due to the genetic and clinical heterogeneity of similar disorders, a definitive diagnosis of KFSD is often daunting. We report a case series from India of three siblings, including a young girl and her two elder brothers, all of whom had features consistent with KFSD. There morphological heterogeneity and differential diagnosis of the condition is also discussed.

Keywords: Keratosis Pilaris; Follicularis; Keratosis; Alopecia; Cicatricial.