Accumulating evidence suggests an immune dysregulation origin characterized by a predominance of T helper 2 (Th2) cytokines both in the blood of patients[11] and directly into lesions.[12] Mast cells and T cells appear to be responsible for cytokines production.[13] Interleukin 5 (IL-5), IL-4, and IL-13 are thought to be involved in the development of KD as they account for 2 biological parameters i.e. HE, and high serum IgE levels. This evidence concerns the gene IGHE and hereditary elliptocytosis.