Two different cases of complete LOF mutations B cell CLL/lymphoma 10 (BCL10) (61, 62) have been described with a combined immunodeficiency without atopic features, though some patients with IEI involving mucosa-associated lymphoid tissue lymphoma translocation protein 1 (MALT1) paracaspase have presented with severe atopic dermatitis in infancy (63–65). Here, BCL10 is linked to immune system disorder.