Omenn's syndrome and related combined immunodeficiency syndromes: diagnostic considerations in infants with persistent erythroderma and failure to thrive

J Am Acad Dermatol. 1991 Aug;25(2 Pt 2):442-6. doi: 10.1016/0190-9622(91)70225-q.


A 4-month-old male infant had a 2-month history of an exfoliative erythroderma and alopecia. Recurrent mucosal infections, diffuse lymphadenopathy, hepatosplenomegaly, lymphocytosis and eosinophilia, anemia, and failure to thrive later developed. Investigation revealed a combined immunodeficiency with T cells of an unusual phenotype in his peripheral blood, skin, and lymph nodes. Our patient's clinical manifestations most closely resemble Omenn's syndrome, a rare form of autosomal recessive combined immunodeficiency.

Publication types

  • Case Reports

MeSH terms

  • Alopecia / pathology
  • Dermatitis, Exfoliative* / pathology
  • Diagnosis, Differential
  • Failure to Thrive*
  • Humans
  • Immunologic Deficiency Syndromes* / pathology
  • Infant
  • Male
  • Syndrome
  • T-Lymphocytes / pathology