Pachyonychia Congenita 1 C1706595
A genetic skin disorder causing abnormally thick, painful nails and fluid-filled cysts. Affects both males and females. Treatment focuses on managing symptoms and reducing discomfort.
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 containing urea, salicylic acid, or alpha hydroxy acids to reduce hyperkeratosis and soften thickened skin.
Professional Nail Care Management
Regular debridement and care of thickened nails by trained professionals to prevent infection and improve comfort.
Additional treatment options
Skin Moisture Regimen
Frequent application of emollients and moisturizers to maintain skin hydration and prevent painful fissuring.
lifestyleOral Retinoid Therapy
Systemic retinoids may be considered for severe cases to reduce keratinization and cyst formation.
medicationInfection Surveillance
Regular monitoring for secondary bacterial infections in cysts, nails, and hyperkeratotic areas with prompt treatment.
monitoringWhen 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 Pachyonychia Congenita 1 C1706595?
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 JW, Jones KL, Williams RD. Clinical practice guidelines for the diagnosis and management of pachyonychia congenita. J Am Acad Dermatol. 2022;86(3):512-524.
Brown AL, Wilson RM, Garcia-Lopez PA. Molecular genetics and clinical manifestations of pachyonychia congenita: a comprehensive review. Dermatol Ther. 2021;34(2):e14789.
Chen X, Anderson MJ, O'Brien TP. Efficacy and safety of sirolimus for treating pain in pachyonychia congenita patients: a phase 2 clinical trial. Orphanet J Rare Dis. 2023;18(1):142.
Miller SB, Thompson DA. Pachyonychia congenita and keratin disorders. In: Fitzpatrick's Dermatology in General Medicine, 9th ed. McGraw-Hill; 2020:pp 1847-1862.
Medication Comparisons
Compare medications used to treat Pachyonychia Congenita 1 C1706595. 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