Ask The Expert
January 8, 2014
When should a child with repeated skin infection of moderate to severe atopic dermatitis (4 to 6 courses of antibiotics annually), but with no infections elsewhere, be investigated for immunodeficiency?
By Dr. Punchama Pacharn and Prof. Pakit Vichyanond:
Atopic dermatitis (AD) is a chronic inflammatory disease with defects of the innate immune system, including in the production of antimicrobial peptides, diminished recruitment of cells to the skin, and epidermal barrier abnormalities (1). Furthermore, the skin colonization of S. aureus and Malassezia species has been reported (2, 3). Therefore, AD patients are susceptible to recurrent skin infections from S. aureus, fungus, and several viruses (3, 4). Severe systemic infections with Herpes simplex, i.e., disseminated eczema herpeticum (EH) could occur in AD patients (3). This group of patients has more severe symptoms, higher numbers of circulating eosinophil, higher levels of serum CCL17, higher incidents of asthma and a higher specific IgE sensitization to inhaled and food allergens (5).
However, most infections in AD patients would require systemic antibiotic treatment in less than 50%, and are predisposed to EH in less than 5% (6). Therefore, an evaluation for immunodeficiency should be considered when patients have severe or difficult to treat skin infections or those with infections in organ systems other than skin.
Primary immunodeficiency diseases (PIDD) with eczema as an associated manifestation include hyper IgE syndrome, Wiskott-Aldrich syndrome, Omenn’s syndrome, and immunodysregulation polyendocrinopathy enteropathy X-linked syndrome (IPEX). These patients present with distinctive clinical characteristics that can lead to proper diagnosis. These are as follows:
- Hyper IgE syndrome: eczema, high IgE, eosinophilia similar to AD patients, coarse facies, and musculoskeletal abnormalities. Severe infections such as internal organ abscess of lungs and liver (7).
- Wiskott-Aldrich syndrome (WAS), other than eczema, most WAS patients have thrombocytopenia, small platelets, and combined immunodeficiency that could cause recurrent or severe infections.
- Omenn’s syndrome is a form of severe combined immunodeficiency disease (SCID) with clinical signs such as recurrent pneumonia, diarrhea, and candida infections. In addition, they frequently have hepatosplenomegaly and lymphadenopathy.
- IPEX syndrome is characterized by a triad of enteropathy, endocrinopathy (diabetes or thyroid disease), and dermatitis. Most patients suffer from severe diarrhea and failure to thrive.
In conclusion, an evaluation for immune deficiency in AD patients with severe infections in organ systems other than the skin should be carried out. In addition, clinical characteristics of distinct PIDD syndrome with eczema should be considered.
- De Benedetto A, Agnihothri R, McGirt LY, Bankova LG, Beck LA. Atopic dermatitis: a disease caused by innate immune defects? J Invest Dermatol. 2009 Jan;129(1):14-30.
- Boguniewicz M, Leung DY. Recent insights into atopic dermatitis and implications for management of infectious complications. J Allergy Clin Immunol. 2010 Jan;125(1):4-13; quiz 4-5.
- Ong PY, Leung DY. The infectious aspects of atopic dermatitis. Immunol Allergy Clin North Am. 2010 Aug;30(3):309-21.
- Wollenberg A, Wetzel S, Burgdorf WH, Haas J. Viral infections in atopic dermatitis: pathogenic aspects and clinical management. J Allergy Clin Immunol. 2003 Oct;112(4):667-74.
- Beck LA, Boguniewicz M, Hata T, Schneider LC, Hanifin J, Gallo R, et al. Phenotype of atopic dermatitis subjects with a history of eczema herpeticum. J Allergy Clin Immunol. 2009 Aug;124(2):260-9, 9 e1-7.
- Leung DY. New insights into atopic dermatitis: role of skin barrier and immune dysregulation. Allergol Int. 2013 Jun;62(2):151-61.
- Schimke LF, Sawalle-Belohradsky J, Roesler J, Wollenberg A, Rack A, Borte M, et al. Diagnostic approach to the hyper-IgE syndromes: immunologic and clinical key findings to differentiate hyper-IgE syndromes from atopic dermatitis. J Allergy Clin Immunol. 2010 Sep;126(3):611-7 e1.
Dr Punchama Pacharn, MD
Professor Pakit Vichyanond, MD
Division of Allergy and Immunology
Department of Pediatrics
Faculty of Medicine Siriraj Hospital
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