Keratosis follicularis spinulosa decalvans

Orpha code: 2340OMIM code: 604093

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.

Disease data
Classification

Disease

ORPHA code
2340
OMIM code
604093
ICD10 code
Q82.8
ICD11 code
ED56

No additional description.

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