Back issues No.2 - 2011 / Case Report  

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Omenn syndrome: a case report and review of literature
Chia-Chi Hsu , Julia Yu-Yun Lee , Sheau-Chiou Chao

Exfoliative dermatitis or erythroderma in infancy is rare. Clinicians need to be alert to the possible
diagnosis of Omenn syndrome (OS), a rare form of combined immunodeficiency in infants presenting
with recurrent infections, erythroderma, lymphadenopathy, hepatosplenomegaly, eosinophilia, and
increased serum IgE levels. OS is fatal unless treated by hematopoietic stem cell transplantation.
We described a 26-day-old boy who had presented with diffusely thick scales on the scalp and some
pustules on his face and trunk since birth. The rash was initially treated as presumed seborrheic
dermatitis. At the age of 42 days, he developed extensive exfoliative dermatitis, lymphadenopathy,
hepatosplenomegaly, and failure to thrive as well as eosinophilia and increased serum IgE levels. The
histopathology of the skin and lymph node were consistent with the diagnosis of OS. He had been
prepared for allograft bone marrow transplantation twice at 5 months and 7 months of age, but the
procedure was not performed because of catheter-related sepsis. The patient eventually died of sepsis
secondary to perianal cellulitis at 20 months of age.

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