Preferred Label : Keratosis follicularis spinulosa decalvans;
ICD-11 definition : 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.;
Origin ID : 303213910;
Automatic exact mappings (from CISMeF team)
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.
