Hyper Ige Syndrome 6 Autosomal Dominant With Recurrent Infections C5848786
An inherited immune defect with very high antibody levels and recurrent bacterial infections of skin and lungs. Patients may also have skeletal and dental abnormalities. Management requires ongoing medical care.
Key facts at a glance
- ICD-10
- D89.9
- ICD-11
- 3A00.0
- Body system
- immunologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Recurrent bacterial pneumonia
Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Eczematous dermatitis
(immunologic)Cold abscesses
(immunologic)Retained primary teeth
(immunologic)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
Immunoglobulin Replacement Therapy
Regular intravenous or subcutaneous immunoglobulin infusions to provide passive immunity and compensate for the inability to produce specific antibodies. This helps prevent recurrent bacterial and fungal infections typical of the syndrome.
Targeted drug therapy with dose adjustment
Active treatment with regular monitoring by a healthcare provider is recommended.
Antibiotic Prophylaxis
Long-term prophylactic antibiotics such as trimethoprim-sulfamethoxazole to prevent Pneumocystis jirovecii pneumonia and other opportunistic bacterial infections common in immunocompromised patients.
Additional treatment options
Regular Infection Surveillance
Frequent clinical monitoring with periodic laboratory assessment including complete blood counts, immunoglobulin levels, and imaging studies to detect and address infections early before complications develop.
monitoringInfection Prevention Practices
Implementation of rigorous hygiene measures including frequent handwashing, avoidance of crowded environments during outbreaks, and careful food preparation to minimize exposure to potential pathogens.
lifestyleNutritional Support
Comprehensive nutritional assessment and supplementation as needed to support immune function, with particular attention to adequate protein intake and micronutrient levels that may be depleted during chronic infections.
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 Hyper Ige Syndrome 6 Autosomal Dominant With Recurrent Infections C5848786?
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.
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