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Keratosis pilaris atrophicans

Keratosis pilaris atrophicans (KPA) represents a group of rare genodermatoses characterized by perifollicular keratosis and inflammation that progresses to atrophy and scarring of the facial skin. Keratosis pilaris of extensor surfaces of limbs is a common associated finding. Affected individuals may present with features that overlap between 3 subtypes, keratosis pilaris atrophicans faciei (KPAF), keratosis follicularis spinulosa decalvans (KFSD), and atrophoderma vermiculata (AVA; see 209700) (summary by Klar et al., 2015). [from OMIM]

MedGen UID:
Concept ID:
Disease or Syndrome

Abnormal perifollicular morphology

Any structural anomaly in the areas surrounding the hair follicles. [from HPO]

MedGen UID:
Concept ID:
Anatomical Abnormality

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