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Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis
Published on: December 15, 2011
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[Hyper IgE syndrome: atopic dermatitis as first manifestation. Case report]
Mayne M Carrera Dangond1, Astrid Schellnast Faure2, M Fernanda Vargas3
1Residencia de Pediatría, Hospital de Niños Sor María Ludovica, La Plata, Argentina.
Archivos Argentinos De Pediatria
|July 26, 2021
Summary
Autosomal dominant hyper-IgE syndrome can present with severe eczema in infants. Early suspicion is crucial for diagnosing this primary immunodeficiency in babies with skin issues.
Area of Science:
- Pediatric Dermatology
- Immunology
- Genetics
Background:
- Atopic dermatitis, a common eczema, typically appears in infancy.
- Diagnosing severe eczema, especially with atypical presentations or infections, poses challenges for pediatricians.
- Primary immunodeficiencies require high suspicion in infants with severe eczema.
Observation:
- This paper details a case of a 1-month-old infant girl with congenital skin involvement.
- The infant was diagnosed with autosomal dominant hyper-IgE syndrome.
- This condition involves recurrent infections, atopic dermatitis, eosinophilia, and elevated IgE.
Findings:
- The infant presented with severe skin manifestations from birth.
- Diagnostic workup confirmed autosomal dominant hyper-IgE syndrome.
- This highlights a rare but significant cause of severe infantile eczema.
Implications:
- Early diagnosis of hyper-IgE syndrome is vital for timely management.
- Recognizing primary immunodeficiencies associated with severe eczema improves patient outcomes.
- This case underscores the importance of considering genetic disorders in infantile eczema.
