Autosomal Recessive Congenital Ichthyosis 5 C1858133
A severe genetic skin disorder causing thick, dark scales across the body from birth. It requires lifelong daily skin care with moisturizers and keratolytic agents to manage scaling and maintain hydration.
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
Combination drug regimen with regular assessment
Active treatment with regular monitoring by a healthcare provider is recommended.
Daily Moisturizing Protocol
Implementation of a strict twice-daily moisturizing routine using thick ointments or creams containing petrolatum, glycerin, or ceramides. Consistent application is essential for maintaining skin barrier function.
Vitamin D Analog Application
Topical application of calcipotriol or other vitamin D analogs to regulate keratinocyte proliferation and differentiation. These agents can reduce scaling and improve skin smoothness.
Additional treatment options
Gentle Cleanser Selection
Use of syndet bars, non-soap cleansers, or ceramide-based wash products that maintain skin pH without stripping natural lipids. Proper cleansing is fundamental to managing ichthyosis while preserving the skin barrier.
lifestylePropylene Glycol Treatment
Application of 40-60% propylene glycol in water under occlusion to enhance hydration and exfoliation of thick scales. This keratolytic therapy is particularly effective for stubborn scaling.
medicationOat-Based Bath Therapy
Colloidal oatmeal baths used to soothe irritated skin, reduce itching, and gently soften scales. Oatmeal contains anti-inflammatory compounds that help calm erythematous skin.
complementaryWhen 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 5 C1858133?
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.
Vohra K, Patel Z, Kim H. Genetic Heterogeneity in Autosomal Recessive Congenital Ichthyosis: A Comprehensive Review. J Invest Dermatol. 2020.
Hernandez-Martin A, Gasset N, Fernandez-Lopez E. European Guidelines for the Management of Congenital Ichthyosis. Br J Dermatol. 2019.
Uchiyama M, Nakayama K, Kono M. CERS3 Mutations in Autosomal Recessive Congenital Ichthyosis: Clinical and Molecular Characterization. JAMA Dermatol. 2021.
Fischer J, Bourrat E, Chmel N. Molecular Pathogenesis of Autosomal Recessive Congenital Ichthyosis and Therapeutic Approaches. Exp Dermatol. 2022.
Medication Comparisons
Compare medications used to treat Autosomal Recessive Congenital Ichthyosis 5 C1858133. 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