Rybojad et al. (1996) described a newborn male, born to consanguineous parents, who manifested nephrotic syndrome in addition to typical findings of Omenn syndrome. Renal biopsy confirmed minimal change glomerular disease.

Elnour et al. (2007) reported a 6-week old infant, born to consanguineous parents, who was referred with generalized lymphadenopathy and gross hepatomegaly of three weeks duration. Upon physical examination, the child was found to be febrile, with generalized lymphadenopathy, edema of the face and extremities, hepatosplenomegaly, and diffuse erythrodermic, scaly icthyotic rashes over the entire body. Blood tests showed a high esinophil count at 17.4 x 10^9/l, extremely high serum IgE levels (25200 kiu/l), and low levels of all other immunoglobulins. Flow cytometry detected a virtual absence of B cells marked by anti CD20, while all lymphocytes were CD3+. The absence of B and preponderance of T cells was confirmed by bone marrow aspiration. Loss of follicular architecture was noticed in the histopathological examination of the lymph nodes. Skin biopsy showed epidermal parakeratosis, moderately heavy lymphohistiocytic infiltrate in the dermis, and marked exocytosis. The child was treated with broad spectrum antibiotics and immunoglobulin infusions. However, he died at the age of four months from septicemia and septic shock.

[Elnour IB, Ahmed S, Halim K, Nirmala V. Omenn's syndrome. Sultan Qaboos Univ Med J. 2007; 7(2):63-8.]

El-Araby et al. (1998) reported the first case of Omenn syndrome from Qatar. The patient was a Qatari neonate, born to consanguineous parents from a highly inbred tribe. At 15 days of age, he had oral thrush, followed by maculopapular scaly lesion which grew to cover the whole body. Upon examination at the age of 40-days, he had persistent diarrhea and vomiting, with peeling of skin all over the body. He showed a failure to thrive. A preliminary diagnosis of Leiner's disease was made. Immunologic work up revealed a severe state of combined immunodeficiency, along with a striking elevation in the IgE levels (>4000 IU/ml), and marked eosinophilia (77%). HLA of the mother and child were non-identical, ruling out SCID with Graft Versus Host Disease. The diagnosis of Omenn syndrome was, thus, most likely. Blood culture about a month after admission revealed P.aeroginosa and C.albicans, and he was put on antibiotics. The patient had been given BCG at birth, and was, therefore, scheduled for an emergency bone marrow transplant. However, he succumbed before this could be performed to multiple organ failure and acute respiratory distress at the age of 2 and half months.

Al-Hammadi 2015 described the case of an Emirati newborn female with an unusual presentation of presumptive intestinal obstruction that was subsequently discovered to be Omenn syndrome. The diagnosis was confirmed due to an unspecified homozygous mutation in the RAG1 gene.