Dermatitis herpetiformis is a rare disease that should be considered the cutaneous expression of a gluten-sensitive enteropathy indistinguishable from celiac disease. Dermatitis herpetiformis is often misdiagnosed and to date no guidelines for the management of dermatitis herpetiformis have been published in Literature. The present guidelines have been prepared for dermatologists by the Group for Cutaneous Immunopathology of the Italian Society of Dermatology and Venereology. They reflect the best data available at the time of preparation and the clinical experience of the authors and the members of the Italian Group for Cutaneous Immunopathology. The diagnosis of dermatitis herpetiformis is established clinically, histologically, immunopathologically and serologically. A gluten-free diet (GFD) is the treatment of choice for patients with dermatitis herpetiformis. Dapsone and/or other drugs should be used during the period until the GFD is effective. In conclusion, the present guidelines provide evidence-based guidance for the diagnosis and treatment of dermatitis herpetiformis.