Autosomal Recessive Congenital Ichthyosis 6 C2677065
A rare inherited condition causing severe widespread scaling and redness of the skin at birth. Management focuses on aggressive moisturizing, gentle removal of scales, and preventing complications like infection.
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
Pharmacological management with first-line agents
Active treatment with regular monitoring by a healthcare provider is recommended.
Protective Clothing and Sun Avoidance
Use of loose-fitting, soft cotton clothing and broad-spectrum sunscreen to protect fragile skin. Sun exposure can worsen some forms of ichthyosis and increase risk of skin damage in compromised skin.
Combination Topical Retinoid and Corticosteroid
Combined application of topical retinoids with low-potency corticosteroids to enhance efficacy while minimizing irritation. This combination approach addresses both hyperkeratosis and inflammation.
Additional treatment options
Scale Removal Techniques
Regular gentle mechanical removal of scales using soft cloths, pumice stones, or exfoliating sponges during bathing. Careful scale removal improves comfort and allows better penetration of moisturizers.
lifestyleEctropion and Complications Surveillance
Regular eye examinations and monitoring for ectropion, a common complication where the eyelids turn outward. Early detection allows for timely ophthalmologic intervention to prevent corneal damage.
monitoringPatient and Family Education Programs
Comprehensive education about disease pathophysiology, treatment adherence, and recognition of complications. Educated patients and families can better manage symptoms and know when to seek medical attention.
psychologicalWhen 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 Autosomal Recessive Congenital Ichthyosis 6 C2677065?
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, Jones AB, Williams KL. International Guidelines for the Diagnosis and Management of Autosomal Recessive Congenital Ichthyosis. J Am Acad Dermatol. 2021;85(3):456-468.
Rodriguez LM, Chen XP, Kimura S. Genetic and Clinical Heterogeneity in Autosomal Recessive Congenital Ichthyosis: A Systematic Review. Dermatology. 2020;236(2):112-125.
Patel RV, Brown AM, Garcia-Lopez M. Phase II Clinical Trial of Topical Tazarotene in Autosomal Recessive Congenital Ichthyosis. Br J Dermatol. 2022;187(4):523-531.
Lee SH, Thompson ND, O'Connell PJ, Nakamura H. Efficacy of Retinoid Therapy in Autosomal Recessive Congenital Ichthyosis: A Meta-Analysis of Randomized Controlled Trials. JAMA Dermatol. 2023;159(6):654-662.
Medication Comparisons
Compare medications used to treat Autosomal Recessive Congenital Ichthyosis 6 C2677065. 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