Dermatitis herpetiformis (DH) is an autoimmune bullous disease characterized by intensely pruritic, chronic, and recurrent vesicles on extensor surfaces such as the elbows, knees, and buttocks. The assortment of neutrophils at the papillary tips is the commonplace histopathological finding, and a trademark analytic element is granular immunoglobulin A statement in the papillary dermis by direct immunofluorescence. DH is firmly connected with gluten touchy enteropathy and is viewed as a cutaneous indication of gluten affectability; i.e., an extra-intestinal show of celiac illness. Gluten free eating routine is the primary line treatment for patients with DH and dispone is likewise compelling. DH specially influences Caucasians who convey human leukocyte antigen (HLA)- DQ2 or HLA-DQ8. The major autoantigen is epidermal transglutaminase. This survey centers around the affirmed highlights of DH and our new discoveries explicit to DH in Japanese patients.