Keratolytic winter erythema
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.
L53.9 – Erythematous condition, unspecified
239064000 – Keratolytic winter erythema
- Focal palmoplantar peeling
- Dyshidrotic eczema
- Epidermolysis bullosa simplex
- Tinea pedis
- Pitted keratolysis
- Erythrokeratodermia variabilis (EKV): fixed scaly plaques and figurate erythema are seen on trunk and extremities. Around half of patients with EKV have an associated diffuse palmoplantar keratoderma.
- Peeling skin syndrome
- Inherited palmoplantar keratodermas, including Unna-Thost disease and Vorner disease
- Inflammatory keratodermas, including palmoplantar psoriasis, lichen planus, or discoid lupus