Omenn syndrome

Omenn syndrome is a rare inflammatory condition associated with severe combined immunodeficiency (SCID). It is characterized by widespread skin redness (erythroderma), peeling of the skin (desquamation), hair loss (alopecia), chronic diarrhea, failure to thrive, swollen lymph nodes (lymphadenopathy), and an enlarged liver and spleen (hepatosplenomegaly). This condition affects the immune system, making it hard for the body to fight off infections. The changes seen in the skin and other parts of the body are a result of the immune system working in an abnormal way. People with Omenn syndrome often need careful medical evaluation and monitoring because of the complex nature of this disorder.

People with Omenn syndrome typically experience red and peeling skin, hair loss, and chronic diarrhea. They may also have trouble growing normally, swollen lymph nodes, and an enlarged liver and spleen.

Omenn syndrome is linked to severe combined immunodeficiency (SCID) and has a genetic cause. Variants in genes such as RAG1, RAG2, and DCLRE1C are known to be associated with this condition.

Information about treatment is currently limited for this condition.

Information about prognosis is currently limited for this condition.

Information about research is currently limited for this condition.