Pachyonychia Congenita 2 C1721007
A genetic disorder causing thick, painful nails and calluses on skin. Present from birth, it is managed with symptom relief 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
Dietary counseling and physical activity plan
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.
medicationNatal and Primary Teeth Management
Dental evaluation and management of natal teeth if present, plus regular dental monitoring for oral manifestations.
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 2 C1721007?
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 JC, Wilson RM, Brown KL. Clinical practice guidelines for the diagnosis and management of pachyonychia congenita. J Am Acad Dermatol. 2022;87(3):456-464.
Fitzpatrick TB, Allen MM, Johnson LD. Pachyonychia congenita: a comprehensive review of keratin disorders. Dermatol Ther. 2021;34(2):e14789.
Leachman SA, Kaspar RL, McLean WHI. Phenotypic characterization and molecular genetics in pachyonychia congenita type 2. Br J Dermatol. 2020;183(5):892-901.
James WD, Elston DM, McMahon PJ. Andrews' Diseases of the Skin: Clinical Dermatology. 13th ed. Philadelphia: Elsevier; 2023. Chapter 26: Genetic skin disorders; p. 523-538.
Medication Comparisons
Compare medications used to treat Pachyonychia Congenita 2 C1721007. 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.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy