keratolytic winter erythema
Findings
No curated finding names keratolytic winter erythema yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
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.
Definition from the Mondo Disease Ontology (MONDO:0007854), read 2026-09-29. CC BY 4.0.
- Inheritance
- Autosomal dominant inheritance
- Onset and course
- Juvenile onset · Childhood onset
HPO, annotations 2026-09-02
Features
5 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- ErythemaHPOHP:0010783
- Very frequent (80% to 99% of cases)
- HyperhidrosisHPOHP:0000975
- Occasional (5% to 29% of cases)
- PustuleHPOHP:0200039
- Occasional (5% to 29% of cases)
- Palmoplantar hyperhidrosisHPOHP:0007410
- Palmoplantar peelingHPOHP:0025819
Genes
1 gene
Germline variants in the gene encoding each protein, as classified by GenCC submitters, each in their own words. Limited, disputed and refuted submissions are listed too.
- CTSBHGNC:2527
- Strong · Labcorp Genetics (formerly Invitae) · Autosomal dominant · 2019
- Limited · Ambry Genetics · Autosomal dominant · 2024
- Limited · G2P · Autosomal dominant · 2025
Where it sits
- A kind of
Other names
2 names
Resolves to: keratolytic winter erythema
- Also called
- Erythrokeratolysis hiemalisOudtshoorn disease