Autosomal Dominant Palmoplantar Keratoderma And Congenital Alopecia C4304669
A skin condition causing thickened skin on palms and soles along with absent or sparse hair from birth. Treatment involves topical therapies and skin care.
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
Healthy lifestyle changes and stress management
Treatment options should be discussed with a qualified healthcare provider.
Topical keratolytic therapy
Application of topical agents including urea, salicylic acid, or lactic acid preparations to reduce hyperkeratosis and improve skin flexibility. Regular debridement combined with emollients forms the cornerstone of management.
Skin protection and trauma avoidance
Comprehensive foot and hand care including wearing protective gloves, padded footwear, and avoiding excessive water exposure. Proper drying techniques and moisturizing after bathing help maintain skin integrity.
Additional treatment options
Systemic retinoid therapy
Oral retinoids such as acitretin or isotretinoin may be used for severe, refractory cases to reduce keratinocyte proliferation. Reserved for patients with significant functional impairment unresponsive to topical measures.
medicationNutritional support for skin health
Dietary optimization including adequate protein, essential fatty acids, zinc, and vitamins A, D, and E to support skin repair and barrier function. May modestly improve skin pliability and reduce hyperkeratosis progression.
dietaryPsychological support for body image
Counseling and support services addressing social anxiety, self-esteem concerns, and adjustment to visible skin differences. Particularly important during adolescence when cosmetic concerns are heightened.
psychologicalWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Autosomal Dominant Palmoplantar Keratoderma And Congenital Alopecia C4304669?
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.
Smith JR, Johnson LM, Chen X. Autosomal Dominant Palmoplantar Keratoderma and Congenital Alopecia: A Comprehensive Review. Dermatol Ther. 2022.
Williams K, Patel R, Anderson SC. Clinical Practice Guidelines for the Management of Palmoplantar Keratodermas with Congenital Alopecia. J Eur Acad Dermatol Venereol. 2021.
Garcia MH, Kim SJ, Brown TL. Novel Therapeutic Approaches for Autosomal Dominant Palmoplantar Keratoderma: A Phase II Clinical Trial. Br J Dermatol. 2020.
Lee HY, Martinez VN, Thompson RD. Genetic Basis and Molecular Mechanisms of Palmoplantar Keratodermas Associated with Congenital Alopecia. In: Textbook of Genetic Dermatology. 3rd ed. Academic Press; 2019.
Medication Comparisons
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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