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What is Omenn syndrome represents which of the following immunological condition? and explain in detail?
Omenn syndrome consists of a subset of T cell deficiencies that present with early-onset erythroderma, alopecia, hepatosplenomegaly, and failure to thrive. This peculiar syndrome is due to the hypomorphic mutations in genes usually associated with SCID, i.e., RAG-1, RAG-2,. The patients are very fragile, requiring simultaneous anti-infective therapy, nutritional suppo, and immunosuppression. Ref: Harrisons Principles of Internal Medicine, 18th Edition, Page 2701.