Alopecia Congenita Keratosis Palmoplantaris C1863093
A rare inherited condition featuring hair loss from birth combined with thickened skin on the palms and soles. It follows an autosomal recessive inheritance pattern. Treatment focuses on managing skin symptoms with moisturizers and keratolytic agents.
Key facts at a glance
- ICD-10
- Q84.9
- ICD-11
- LA00.0
- Body system
- dermatologic
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Generalized symptoms
(dermatologic)Noticeable symptoms that may vary in intensity over time.
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
Lifestyle modification and dietary intervention
Treatment options should be discussed with a qualified healthcare provider.
Topical Keratolytic Agents
Application of salicylic acid or urea-based preparations to reduce hyperkeratosis on palms and soles. Regular use helps soften thickened skin and improve comfort.
Emollients and Barrier Creams
Regular application of thick moisturizers and occlusive barrier preparations to maintain skin hydration and reduce fissuring. Should be applied frequently, especially after hand washing.
Additional treatment options
Systemic Retinoids
Oral retinoids such as acitretin to reduce keratinocyte proliferation and improve skin scaling. Reserved for moderate to severe cases unresponsive to topical therapy.
medicationExcimer Laser Phototherapy
Targeted UVB laser treatment to affected skin areas to reduce hyperkeratosis and improve skin texture with precise dosing.
procedureGenetic Counseling
Comprehensive genetic evaluation and family counseling to understand inheritance patterns, disease prognosis, and available reproductive options.
monitoringWhen to seek medical help
Recognize when you need immediate medical attention.
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Frequently asked questions
Common questions about this topic.
What is Alopecia Congenita Keratosis Palmoplantaris C1863093?
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.
Martinez-Reyes JR, Patel SN. Congenital alopecia with palmoplantar keratoderma: A comprehensive review of clinical presentations. Orphanet J Rare Dis. 2019.
Tanaka H, Schmidt MW, European Dermatology Network Consortium. Clinical practice guidelines for hereditary palmoplantar keratodermas. Br J Dermatol. 2021.
Okonkwo R, Fernandez Garcia KL. Novel mutations in keratins associated with congenital alopecia-keratosis syndrome. J Eur Acad Dermatol Venereol. 2018.
Volk AE, Kunitz C. Genetic and clinical heterogeneity in palmoplantar keratodermas: A systematic review and meta-analysis. Am J Med Genet A. 2022.
Medication Comparisons
Compare medications used to treat Alopecia Congenita Keratosis Palmoplantaris C1863093. 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.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy