alt=Redness and scaliness of the entire skin on a 5-month-old female infant The symptoms are very similar to graft-versus-host disease (GVHD). This is because the patients have some T cells with limited levels of recombination with the mutant RAG genes. These T cells are abnormal and have a very specific affinity for self antigens found in the thymus and in the periphery. Therefore, these T cells are auto-reactive and cause the GVHD phenotype. A characteristic symptom is chronic inflammation of the skin, which appears as a red rash (early onset erythroderma). Other symptoms include eosinophilia, failure to thrive, swollen lymph nodes, swollen spleen, diarrhea, enlarged liver, low immunoglobulin levels (except immunoglobulin E, which is elevated), low T cell levels, and no B cells.
Omenn syndrome
Wildervanck syndrome
Malouf syndrome
Hanhart syndrome
Aagenaes syndrome
Gleich's syndrome
Kocher–Debre–Semelaigne syndrome
Pelger–Huët anomaly