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Articles
Published: 2023-05-27

Omenn syndrome: the drama of a family, congenital ichthyosis is not always mundane!

Mother and Child Department, University Hospital Mohamed VI, Faculty of Medicine And Pharmacy of Oujda, Morocco
Mother and Child Department, University Hospital M ohamed VI, Faculty of Medicine And Pharmacy of Oujda, Morocco
Mother and Child Department, University Hospital Mohamed VI, Faculty of Medicine And Pharmacy of Oujda, Morocco
Mother and Child Department, University Hospital Mohamed VI, Faculty of Medicine And Pharmacy of Oujda, Morocco
Mother and Child Department, University Hospital Mohamed VI, Faculty of Medicine And Pharmacy of Oujda, Morocco
Mother and Child Department, University Hospital Mohamed VI, Faculty of Medicine And Pharmacy of Oujda, Morocco
Mother and Child Department, University Hospital Mohamed VI, Faculty of Medicine And Pharmacy of Oujda, Morocco
Omenn Syndrome, Ichthyosis, Severe Combined Immunodeficiency, Congenital, Morocco

Abstract

Background: The case we are reporting is about one of the rare manifestations of severe combined immunodeficiency, Omenn syndrome (OS).

Case presentation: A 43-days-old female presented with thick diffuse erythrodermic scaly ichthyosiform lesions on the scalp, face, and trunk since birth. lymphadenopathy, splenomegaly, and growth retardation as well as eosinophilia and increased serum IgE levels. A pregnancy was planned for an allograft of bone marrow, but the procedure was not carried out due to a persistent post-covid pneumopathy with bilateral parenchymal condensation that resulted in death.

Conclusion: This case report intends to incite clinicians to be alert to this possible diagnosis and not to underrate an immune deficiency in the case of neonatal erythroderma.



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References

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How to Cite

1.
Bouhmidi M, Hajar B, Ghannam A, Aziza E ouali, Babakhouya A, Rkain M, Benajiba N. Omenn syndrome: the drama of a family, congenital ichthyosis is not always mundane!. jidhealth [Internet]. 2023 May 27 [cited 2024 May 23];6(2):861-3. Available from: https://jidhealth.com/index.php/jidhealth/article/view/282