Combined Immunodeficiency With Skin Granulomas C2673536
A rare immune disorder where the body cannot fight infections properly and develops skin nodules called granulomas. Patients typically need infection prevention and immune system support. Severity varies among individuals.
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 infections
Autoimmune manifestations
Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Granulomatous skin lesions
(skin)Failure to thrive
(general)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.
Immunoglobulin Replacement Therapy
Regular intravenous or subcutaneous immunoglobulin infusions to compensate for antibody deficiency and reduce susceptibility to recurrent infections in combined immunodeficiency.
Hematopoietic Stem Cell Transplantation
Curative approach through allogeneic stem cell transplantation to reconstitute a functional immune system, potentially resolving both immunodeficiency and skin granuloma manifestations.
Additional treatment options
Antimicrobial Prophylaxis
Prophylactic antibiotics and antifungals to prevent opportunistic infections, particularly important in patients awaiting or not candidates for transplantation.
medicationSkin Granuloma Management
Dermatological procedures including intralesional corticosteroid injections, cryotherapy, or surgical excision of symptomatic granulomas, coordinated with systemic immune therapy.
procedureImmunological and Dermatological Surveillance
Regular monitoring of immune parameters, infection frequency, and skin granuloma status with periodic immunological reassessment to guide ongoing therapy adjustments.
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 Combined Immunodeficiency With Skin Granulomas C2673536?
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.
Schmidt JW, Anderson MK, Patel PB. International consensus guidelines for diagnosis and management of combined immunodeficiency with granulomatous skin manifestations. Clin Immunol Pract. 2022.
Martinez VF, Thompson LR, Chen SX. Granulomatous complications in primary immunodeficiency disorders: a comprehensive review. Curr Allergy Asthma Rep. 2021.
Yamamoto RK, Fernandez EJ, Lopez MN. Cutaneous granulomas in combined immunodeficiency: clinical features and treatment outcomes in 15 patients. J Clin Immunol. 2020.
Roberts CJ, Kim HA, Santos LS. Skin manifestations in inborn errors of immunity: focus on granulomatous lesions. Immunol Allergy Clin North Am. 2023.
Medication Comparisons
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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.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy