Keratosis follicularis spinulosa decalvans is a rare, X-linked disorder characterized by scarring alopecia of the scalp and eyebrows in the setting of widespread keratosis pilaris. Less frequent associations are ocular abnormalities and palmoplantar keratoderma. Acne keloidalis nuchae has previously been described in one patient with keratosis follicularis spinulosa decalvans. We report another...

1. van Osch LD, Oranje AP, Keukens FM, Voorst Vader van PC, Veldman E. Keratosis follicularis spinulosa decalvans: A family study of seven male cases and six female carriers. J Med Genet 1992;29:36-40. 2. Fong K, Wedgeworth EK, Lai-Cheong JE, Tosi I, Mellerio JE, Powell AM, et al. MBTPS2 mutation in a British pedigree with keratosis follicularis spinulosa decalvans. Clin Exp Dermatol 2012;37:63...

Keratosis follicularis spinulosa decalvans (KFSD) is a hereditary disorder of the hair follicle which presents with scarring alopecia and follicular papules affecting the scalp and other areas of the body. Being X-linked it is more common in males but rarely, can be seen in females. We report this rare disorder in siblings affecting both male and female child. Key-wordsKeratosis follicularis sp...

KFSD: Keratosis folicularis spinulosa decalvans SP: Substance P INTRODUCTION Keratosis follicularis spinulosa decalvans (KFSD) is an inherited rare disorder characterized by diffuse keratosis pilaris and scarring alopecia. Palmoplantar keratoderma, ocular abnormalities, and atopy can also be present. Most cases occur in males and have a X-linked pattern of inheritance, although autosomal domina...

keratosis follicularis spinulosa decalvans (kfsd) represents a rare, probably x-linked recessive genodermatosis, characterized by keratosis pilaris of face, trunk and extremities, followed by atrophy, cicatricial alopecia of the scalp, eyebrows and eyelashes, photophobia and corneal abnormalities. we report a rare case of kfsd and review the literature.

Keratosis follicularis spinulosa decalvans (KFSD) is a rare, X linked disorder with skin and eye involvement (MIM 308800). We have studied a large British family with KFSD using polymorphic markers from Xp21-p23 and obtained a lod score of 2.056 at theta=0 with markers proximal and distal to the KFSD candidate region Xp22.13-p22.2 identified by Oosterwijk et al. Our data confirm the linkage to ...