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Keratosis follicularis spinulosa decalvans is a rare genodermatosis occurring during infancy or childhood, predominantly affecting males, and characterized by diffuse follicular hyperkeratosis associated with progressive cicatricial alopecia of the scalp, eyebrows and eyelashes. Additional findings can include photophobia, corneal dystrophy, facial erythema, and/or palmoplantar keratoderma.
Estimated prevalence: <1 in 1,000,000 (VERY_RARE).
No clinical trials have been registered for keratosis follicularis spinulosa decalvans.
2 publications have been identified in PubMed for keratosis follicularis spinulosa decalvans. Research spans Case Report / Case Series (50%) and Gene Therapy / Novel Therapeutics (50%).
Cuperus E (2026). [PMID: 40960197](https://pubmed.ncbi.nlm.nih.gov/40960197/). *Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG*. [Gene Therapy / Novel Therapeutics]
Brashi R (2024). [PMID: 39205768](https://pubmed.ncbi.nlm.nih.gov/39205768/). *Cureus*. [Case Report / Case Series]
Data assembled from 3 of 12 sources · Last updated Sep 20, 2026, 5:35 PM UTC
European rare disease database
Genetic and Rare Diseases Info Center