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Omenn's syndrome: lessons from a red baby

R P Katugampola1, G Morgan, R Khetan

  • 1Department of Dermatology, School of Medicine, Cardiff University, Cardiff, UK. ruwanik@hotmail.com

Clinical and Experimental Dermatology
|May 24, 2008
PubMed

Insights

Omenn's syndrome (OS) is a rare infant immunodeficiency causing severe skin issues. Early diagnosis and bone marrow transplant (BMT) offer a life-saving treatment for affected infants.

Area of Science:

  • Pediatric Immunology
  • Dermatology
  • Genetics

Background:

  • Omenn's syndrome (OS) is a rare, severe combined immunodeficiency in infants.
  • It presents with erythroderma, exfoliative dermatitis, recurrent infections, eosinophilia, and elevated IgE.
  • OS is typically fatal without timely intervention, primarily bone marrow transplantation (BMT).

Observation:

  • A 3-week-old infant initially presented with a widespread scaly, erythematous rash and stomatitis.
  • Initial diagnoses included atopic eczema and herpes stomatitis.
  • At 3 months, the infant developed erythroderma, diarrhea, and hepatosplenomegaly.

Findings:

  • The infant exhibited eosinophilia, markedly elevated serum IgE, and low IgG, IgA, and IgM levels.
  • Immunological workup revealed abnormal lymphocyte populations.
  • Skin histology was consistent with Omenn's syndrome.
  • The diagnosis was confirmed as Omenn's syndrome.

Implications:

  • This case highlights the importance of recognizing Omenn's syndrome in infants with severe dermatitis and systemic symptoms.
  • Prompt diagnosis and BMT are critical for survival in OS.
  • Successful BMT from an unrelated donor offers a viable treatment option, even in complex cases.

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