Keratolytic winter erythema

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Keratolytic winter erythema

ORPHA:50943Disease

Also called Erythrokeratolysis hiemalis · Oudtshoorn disease

What it is

Keratolytic winter erythema is a rare epidermal disease, characterized by recurrent centrifugal palmoplantar peeling and erythema presenting seasonal variation (cold weather). Skin lesions may spread to the dorsum of hands and feet and to the interdigital spaces. Lower legs, knees and thighs may also be involved. Episodes may be preceded by itch and hyperhidrosis. Skin biopsy reveals an epidermal spongiosis with clefting in the stratum corneum, followed by regrowth. Keratolytic winter erythema follows an autosomal dominant mode of transmission.

Key facts

Age of onset
Adolescent, Adult, Childhood, Infancy, Neonatal
Inheritance
Autosomal dominant
Classified as
Disease

Signs and symptoms

Very common80–99%

1

Sometimes5–29%

2

These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.

Gene

CTSBRole in the phenotype of

ICD-10 codes

L30.8filed under a broader ICD-10 category — shared with 1 other rare disease

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Cross-references

GARD 8275MESH C536155MONDO 0007854OMIM 148370UMLS C0406756

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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