Autoimmune diseases (AI) are pathological conditions characterized by irregular response of immune system to self-antigens. AI diseases are relatively uncommon, affecting around 5% of the population worldwide. The most common AI diseases with oral manifestations are, oral lichen planus, pemphigus vulgaris, paraneoplastic pemphigus, mucous membrane pemphigoid and bullous pemphigoid. Recurrent clinical sign of listed diseases is desquamative gingivitis (DG). DG is most common in individuals between forty and sixty years of age. Women are more often affected than men. Some studies reported few cases appearing in children. DG is a descriptive clinical term used for erythematous gingiva, atrophy, blister formation and desquamation of the gingival epithelium that affect both free and attached gingiva. Diagnosis of the underlying systemic disease depends on results from histopathology and direct immunofluorescence microscopy, results from patient serum for indirect immunofluorescence and other immunoserological tests, and microbiological test for infectious causes. Asymptomatic cases of DG should be treated with improvement of oral hygiene and frequent scaling and root planning. Mild cases should be treated with topical corticosteroids applied with custom build acrylic carriers. Moderate to severe cases should be treated with systemic corticosteroids. Approach to treatment of DG should be multidisciplinary, including general dentist, periodontology and oral medicine specialist, dermatologist, and ophthalmologis