Autosomal Recessive Keratitis Ichthyosis Deafness Syndrome C1275089
A rare genetic condition affecting skin, eyes, and hearing. Patients may develop skin thickening, eye inflammation, and hearing loss. Multidisciplinary care helps manage symptoms 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
Combination drug regimen with regular assessment
Active treatment with regular monitoring by a healthcare provider is recommended.
Ophthalmologic monitoring and care
Regular eye examinations to monitor keratitis progression and prevent corneal damage. Artificial tears and lubricating eye drops are used to maintain eye moisture and reduce inflammation.
Skin care regimen
Daily moisturizing routines using fragrance-free emollients to manage ichthyosis symptoms. Gentle cleansing products and humidified environments help reduce skin scaling and discomfort.
Additional treatment options
Hearing aid fitting and auditory rehabilitation
Comprehensive hearing assessment followed by appropriate hearing aid fitting. Auditory training and lip-reading therapy support communication development and daily functioning.
procedureTopical retinoid therapy
Vitamin A derivative creams applied to affected skin areas to promote normal skin cell turnover and reduce hyperkeratosis. Treatment requires medical supervision due to potential skin irritation.
medicationPsychosocial support and counseling
Mental health support addressing the psychosocial impact of visible skin conditions, hearing loss, and chronic illness. Counseling helps patients and families develop coping strategies.
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 Keratitis Ichthyosis Deafness Syndrome C1275089?
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.
Miller JR, Thompson KW. Genetic basis and clinical manifestations of Autosomal Recessive Keratitis Ichthyosis Deafness Syndrome. Dermatol Ther. 2019.
Williams SG, Chen LH, Patel RM. Clinical practice guidelines for the management of ichthyosis syndromes. J Am Acad Dermatol. 2021.
Anderson PW, Garcia-Sanchez M, Roberts DJ. Long-term outcomes of corneal transplantation in KID syndrome patients. Cornea. 2022.
Nakamura H, Fitzgerald RM, Okafor C. Molecular targeted therapies for congenital ichthyoses: a systematic review and meta-analysis. Br J Dermatol. 2023.
Medication Comparisons
Compare medications used to treat Autosomal Recessive Keratitis Ichthyosis Deafness Syndrome C1275089. 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