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Dermatitis herpetiformis (DH) is a pruritic papulovesicular disease that is relatively common in Caucasian populations, and was first reported by Louis Duhring. It is characterized by granular IgA deposition in the dermal-epidermal junction and gluten-sensitive enteropathy (GSE) associated with human leukocyte antigen-DQ2/DQ8 haplotype. In contrast, DH is rare in Japan, and Japanese DH patients occasionally show unique features, including a high frequency of fibrillar IgA deposition in the papillary dermis, and rare occurrence of GSE. We refer to this condition in Japanese patients as “fibrillar-type DH”, while the DH that is typically observed in the Caucasian population is referred to as “granular-type DH”. In patients with typical fibrillar-type DH, IgA antibodies to epidermal transglutaminase are the only antibodies detected. In addition, Th2-type cytokines, such as interleukin (IL)-4, IL-5 and IL-13, may be important in the development of skin lesions in fibrillar-type DH (especially in the infiltration of tissue by eosinophils), as in granular-type DH. The pathogenesis of fibrillar-type DH may differ from that of granular-type DH, which is dependent on gluten and in which IgA antibodies to tissue transglutaminase (the main antigen in GSE) are detected. We herein review the clinical, histological, and immunological findings of fibrillar-type DH.