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Keratolytic winter erythema
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Keratolytic winter erythema

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Contributors: Susan Burgin MD, Lowell A. Goldsmith MD, MPH
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Synopsis

Keratolytic winter erythema (KWE, also known as erythrokeratolysis hiemalis and Oudtshoorn skin disease) is a rare condition seen predominantly in South Africans from the Eastern Cape region of the country.

KWE is characterized by centrifugal peeling of the palms and soles with associated underlying erythema. In its mildest form, interdigital scaling and erythema and mild peeling only are seen. In active cases, peeling may involve the entire palmar or plantar surface, where it may be bordered by a rim of erythema. Peeling progresses centrifugally from multiple sites on acral skin. Skin may be peeled off in long sheets. In more severe cases, expanding erythematous annuli with a trailing edge of scale may be seen on the extremities and, rarely, the trunk. KWE typically worsens in the winter months. Febrile illness is also reported to flare the disease.

Peeling is painless and the condition is not pruritic. Affected individuals, especially those with concomitant hyperhidrosis, are bothered by the odor of macerated keratin.

KWE is inherited in an autosomal dominant fashion. The founder effect is responsible for the high concentration of cases in the Afrikaans-speaking population of the Oudtshoorn region of the Cape Province in South Africa. A large German kindred with KWE has also been reported. KWE may begin in infancy or childhood and it persists into adulthood, although severity tends to lessen with age.

The exact gene locus is being investigated, and pathogenesis has not yet been fully elucidated.

For more information, see OMIM.

Codes

ICD10CM:
L53.9 – Erythematous condition, unspecified

SNOMEDCT:
239064000 – Keratolytic winter erythema

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Last Updated: 03/29/2017
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Keratolytic winter erythema
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Keratolytic winter erythema : Palms and soles, Skin peeling
Copyright © 2019 VisualDx®. All rights reserved.