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Clinical Cases in Pediatric Skin Cancers pp 45–47Cite as

A Girl with Erythema all Over the Body

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Part of the Clinical Cases in Dermatology book series (CLIDADE)

Abstract

Omenn syndrome is a rare and severe combined immunodeficiency disease, with an early onset, desquamative erythroderma, hepatosplenic lymphadenopathy, eosinophilia, diarrhea, IgE Increased, loss of B lymphocytes, severe damage to T and B lymphocytes. The prognosis of this disease is poor, and death is often caused by organ failure and severe infection. Here we reported a case of omenn syndrome with generalized erythema, and review the literature of omenn syndrome briefly.

Keywords

  • Omenn syndrome
  • Drug hypersensitivity syndrome
  • Netherton syndrome

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Fig. 10.1

References

  1. Brauer PM, Pessach IM, Clarke E, et al. Modeling altered T-cell development with human induced pluripotent stem cells from patients with RAG1 mutations and distinct immunological phenotypes. Blood. 2016;128(6):783–93.

    CAS  CrossRef  Google Scholar 

  2. Matthews AGW, Briggs CE, Yamanaka K, et al. Compound heterozygous mutation of rag 1 leading to Omenn syndrome. PLoS One. 2015;10(4):e0121489.

    CrossRef  Google Scholar 

  3. Gruber TA, Shah AJ, Hernandez M, et al. Clinical and genetic heterogeneity in Omenn syndrome and severe combined immune deficiency. Pediatr Transplant. 2010;13(2):244–50.

    CrossRef  Google Scholar 

  4. Chinn IK, Shearer WT. Severe combined immunodeficiency disorders. Immunol Allergy Clin North Am. 2015;35(4):671–94.

    CrossRef  Google Scholar 

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Author information

Authors and Affiliations

  1. Department of Dermatology, Hunan Children’s Hospital, Changsha, China

    Shu-Zhen Yue, Yang-Yang Luo, Jian-Ping Tang & Bin Zhou

Authors
  1. Shu-Zhen Yue
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  2. Yang-Yang Luo
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  3. Jian-Ping Tang
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  4. Bin Zhou
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Editor information

Editors and Affiliations

  1. Dermatologic Unit, University Hospital of Parma, Parma, Italy

    Francesca Satolli

  2. Dermatology, Hanoi Medical University, Hanoi, Vietnam

    Prof. Michael Tirant

  3. Dermatologie & Allergologie, Städtisches Klinikum Dresden, Dresden, Germany

    Uwe Wollina

  4. Science of Communication, Marconi University, Rome, Roma, Italy

    Torello M. Lotti

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Yue, SZ., Luo, YY., Tang, JP., Zhou, B. (2022). A Girl with Erythema all Over the Body. In: Satolli, F., Tirant, M., Wollina, U., Lotti, T.M. (eds) Clinical Cases in Pediatric Skin Cancers. Clinical Cases in Dermatology. Springer, Cham. https://doi.org/10.1007/978-3-030-93666-2_10

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  • DOI: https://doi.org/10.1007/978-3-030-93666-2_10

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-93665-5

  • Online ISBN: 978-3-030-93666-2

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