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Keratosis follicularis spinulosa decalvans

Synonym(s) -
Prevalence <1 / 1 000 000
Inheritance Autosomal dominant
or X-linked recessive
or Autosomal recessive
Age of onset Childhood
  • Q82.8
  • C0343057
  • C536159
MedDRA -


Disease 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.

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