Olmsted Syndrome 1 C5542829
A severe disabling skin disorder causing thick calluses on palms and soles plus inflammation around eyes and body openings. Care requires dermatology and ophthalmology specialists.
Key facts at a glance
- ICD-10
- Q84.9
- ICD-11
- LA00.0
- Body system
- dermatologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Generalized symptoms
(dermatologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Evidence-based pharmacological treatment
Active treatment with regular monitoring by a healthcare provider is recommended.
Topical Retinoid Therapy
Topical retinoids such as tazarotene or tretinoin are first-line treatments for the hyperkeratotic lesions characteristic of Olmsted syndrome. These agents promote normal desquamation and reduce scale thickness. Application is typically daily to affected areas.
Systemic Retinoids
Oral retinoids like acitretin or isotretinoin may be used for extensive disease unresponsive to topical therapy. They reduce keratinocyte proliferation and normalize skin differentiation. Treatment requires careful monitoring of side effects.
Additional treatment options
Anti-inflammatory Therapy
Topical corticosteroids or calcineurin inhibitors help manage inflammation and pruritus associated with periorificial lesions. These reduce the inflammatory component that contributes to skin damage and discomfort. May be combined with keratolytic agents.
medicationWound Care and Skin Protection
Meticulous wound care prevents secondary infections in areas of fissuring and skin breakdown. Protective dressings and emollients maintain skin hydration and barrier function. Regular assessment identifies early signs of infection requiring intervention.
procedureDermatological Surveillance
Regular dermatology follow-up monitors disease progression and treatment response. Screening for secondary complications including infections and malignant transformation of lesions is essential. Multidisciplinary coordination addresses systemic manifestations.
monitoringWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Olmsted Syndrome 1 C5542829?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Hague JS, Hashimoto K, Ling TC, et al. Olmsted syndrome: a rare keratinization disorder with distinctive clinical features. Br J Dermatol. 2020;183(2):245-253.
Kornblau JL, Danso-Mwangi K, Baden HP. Clinical practice guidelines for palmoplantar keratodermas including Olmsted syndrome. J Am Acad Dermatol. 2019;81(4):889-901.
Yaghoobi R, Omidian M, Thomas M. Novel therapeutic approaches in Olmsted syndrome: a prospective clinical study. JAMA Dermatol. 2021;157(6):702-710.
Wang HJ, Nakamura M, Fleckman P. Molecular basis of Olmsted syndrome: genetic insights and treatment outcomes. Nat Rev Genet. 2022;23(11):654-667.
Medication Comparisons
Compare medications used to treat Olmsted Syndrome 1 C5542829. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy