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April 1994

Keratosis Pilaris AtrophicansOne Heterogeneous Disease or a Symptom in Different Clinical Entities?

Author Affiliations

Subdivision of Pediatric Dermatology University Hospital Rotterdam Sophia Children's Hospital; 60 3015 GJ Rotterdam, the Netherlands; Rotterdam Eye Hospital Rotterdam; Clinical Genetics Center Leiden, the Netherlands

Arch Dermatol. 1994;130(4):500-502. doi:10.1001/archderm.1994.01690040104017

IN this issue of the Archives, Baden and Byers1 describe 21 cases of keratosis pilaris atrophicans (KPA). He considers KPA as one disease entity.1 However, our opinion differs in view of the arguments presented here. Keratosis pilaris (KP) is a skin symptom associated with different diseases, mostly of ectodermal origin. By definition, keratinous plugs are observed in the follicular orifices surrounded by a variable degree of erythema. When the keratotic papules are followed by atrophy, one speaks of KPA. Atrophy is preceded with or without inflammation. There is much confusion on KP and KPA entities, especially because different overlapping syndromes have been described and many synonyms are used in both dermatologic and genetic literature.2-4 In this editorial, we present our opinion and arguments on diseases with KPA.

KERATOSIS PILARIS ATROPHICANS  We distinguish four distinct clinical entities that show KPA. These are keratosis pilaris atrophicans faciei (KPAF), atrophoderma

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