Olmsted syndrome
MONDO:0031421Mondo
Findings
No curated finding names Olmsted syndrome yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
A hereditary palmoplantar keratoderma characterized by the combination of bilateral mutilating transgredient palmoplantar keratoderma and periorificial keratotic plaques.
Definition from the Mondo Disease Ontology (MONDO:0031421), read 2026-09-29. CC BY 4.0.
Features
23 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.
- Abnormal fingernail morphologyHPOHP:0001231
- Very frequent (80% to 99% of cases)
- AnhidrosisHPOHP:0000970
- Very frequent (80% to 99% of cases)
- AnkylosisHPOHP:0031013
- Very frequent (80% to 99% of cases)
- ErythemaHPOHP:0010783
- Very frequent (80% to 99% of cases)
- Palmoplantar hyperhidrosisHPOHP:0007410
- Very frequent (80% to 99% of cases)
- Palmoplantar keratodermaHPOHP:0000982
- Very frequent (80% to 99% of cases)
- Skin fissureHPOHP:0031057
- Very frequent (80% to 99% of cases)
- Sparse hairHPOHP:0008070
- Very frequent (80% to 99% of cases)
- Thickened skinHPOHP:0001072
- Very frequent (80% to 99% of cases)
- Abnormality of the dentitionHPOHP:0000164
- Frequent (30% to 79% of cases)
- Carious teethHPOHP:0000670
- Frequent (30% to 79% of cases)
- HypodontiaHPOHP:0000668
- Frequent (30% to 79% of cases)
Show the remaining 11
- Sensorineural hearing impairmentHPOHP:0000407
- Frequent (30% to 79% of cases)
- Skin ulcerHPOHP:0200042
- Frequent (30% to 79% of cases)
- Abnormal gingiva morphologyHPOHP:0000168
- Occasional (5% to 29% of cases)
- Abnormal oral mucosa morphologyHPOHP:0011830
- Occasional (5% to 29% of cases)
- Abnormality of the tongueHPOHP:0000157
- Occasional (5% to 29% of cases)
- AlopeciaHPOHP:0001596
- Occasional (5% to 29% of cases)
Where it sits
- A kind of
- Narrower terms (3)
Other names
3 names
Resolves to: Olmsted syndrome
- Also called
- mutilating palmoplantar hyperkeratosis with periorificial keratotic plaquespalmoplantar and periorificial keratodermapalmoplantar keratoderma, mutilating, with periorificial keratotic plaques