Keratoderma Ichthyosis Deafness Syndrome Autosomal Recessive C5774200
An inherited form of skin thickening, scaling, and hearing loss. Treatment focuses on skin care, hearing support, and managing complications throughout life.
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
Systemic Retinoids
Oral retinoid therapy such as acitretin or isotretinoin to reduce hyperkeratosis and improve skin manifestations in severe cases of autosomal recessive keratoderma-ichthyosis-deafness.
Targeted drug therapy with dose adjustment
Active treatment with regular monitoring by a healthcare provider is recommended.
Topical Keratolytics
Application of urea-based or salicylic acid preparations to reduce hyperkeratotic lesions and improve skin texture on palms and soles.
Additional treatment options
Auditory Rehabilitation
Evaluation for and provision of hearing amplification devices or cochlear implantation depending on degree and type of hearing loss.
procedureHand Mobility Therapy
Physical therapy interventions including stretching exercises and splinting to maintain hand function and prevent contractures from keratoderma.
physical_therapyMultidisciplinary Monitoring
Regular coordinated care involving dermatology, audiology, and ophthalmology to assess disease progression and treatment response.
monitoringWhen 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 Keratoderma Ichthyosis Deafness Syndrome Autosomal Recessive C5774200?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
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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