Omenn syndrome

A case report and review of literature

Research output: Contribution to journalArticle

1 Citation (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)50-54
Number of pages5
JournalDermatologica Sinica
Volume29
Issue number2
DOIs
Publication statusPublished - 2011 Jan 1

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Exfoliative Dermatitis
Severe Combined Immunodeficiency
Eosinophilia
Immunoglobulin E
Sepsis
Seborrheic Dermatitis
Failure to Thrive
Cellulitis
Hematopoietic Stem Cell Transplantation
Exanthema
Scalp
Serum
Bone Marrow Transplantation
Allografts
Catheters
Lymph Nodes
Parturition
Skin
Infection
Lymphadenopathy

All Science Journal Classification (ASJC) codes

  • Dermatology

Cite this

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title = "Omenn syndrome: A case report and review of literature",
abstract = "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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Omenn syndrome : A case report and review of literature. / Hsu, Chia Chi; Lee, Yu-Yun; Chao, Sheau-Chiou.

In: Dermatologica Sinica, Vol. 29, No. 2, 01.01.2011, p. 50-54.

Research output: Contribution to journalArticle

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