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Updated: Jun 4, 2026

Granulocyte-dependent Autoantibody-induced Skin Blistering
Published on: October 12, 2012
[Granulomatous skin disease in an immunocompromised child]
1Département interhospitalier de dermatologie (CHU Saint-Pierre, CHU Brugmann, hôpital universitaire des enfants Reine-Fabiola), 15 avenue J.-J. Crocq, Brussels, Belgium. jboudart@ulb.ac.be
Insights
Cutaneous granulomas in an immunocompromised child, not CVID, responded well to anti-TNFα therapy. This biotherapy successfully cleared lesions and prevented relapse, offering a new treatment avenue.
Area of Science:
- Immunology
- Dermatology
- Pediatrics
Background:
- Systemic and cutaneous granulomas are linked to immunodeficiency, especially common variable immunodeficiency (CVID).
- Cutaneous granulomas can manifest in immunocompromised individuals.
Observation:
- A pediatric patient with undefined immunodeficiency presented with progressive facial and foot granulomatous lesions.
- Standard laboratory tests ruled out infectious or neoplastic causes.
- Biopsies confirmed granulomas, and corticosteroids were ineffective.
Findings:
- Anti-tumor necrosis factor-alpha (anti-TNFα) therapy led to complete resolution of cutaneous granulomas within 6 months.
- Treatment was continued for an additional 6 months, with no recurrence observed 6 months post-discontinuation.
Implications:
- This case highlights cutaneous granulomas in undefined immunodeficiency beyond CVID.
- Successful anti-TNFα therapy suggests biotherapy as a viable treatment option for such cases.
- Further research into granuloma pathogenesis and targeted therapies in immunodeficiency is warranted.
Background:
The development of systemic and/or cutaneous granulomas associated with immunodeficiency is well established, particularly with common variable immunodeficiency (CVID). We report the case of an immunocompromised child presenting with cutaneous granulomas, predominantly on the face.
Patients And Methods:
A boy aged 6 years and 9 months presenting complex and incompletely defined immunodeficiency presented with gradually worsening cutaneous lesions of granulomatous aspect on his face and right foot. Extensive laboratory tests showed no infectious or neoplastic processes. Biopsies of the lesions confirmed the presence of granulomas. Systemic corticosteroids produced no satisfying improvement and were changed to anti-TNFα. The lesions had completely disappeared after 6 months. Treatment was continued for 6 months, with no relapse 6 months after discontinuation.
Conclusion:
The originality of our case lies in the presentation of cutaneous granulomas, appearing in the context of an undefined immunodeficiency other than CVID, as well as in the therapeutic aspect, with the successful use of biotherapies in such a clinical setting.
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