Pemphigus is an uncommon intraepidermal blistering disease occurring on the skin and mucous membranes. It is caused by autoantibodies to adhesion molecules expressed in the skin and mucous membranes. The cause is unknown. The bullae appear spontaneously and are tender and painful when they rupture. Drug induced pemphigus from penicillamine, captopril, and others have been reported. There are several forms of pemphigus: pemphigus vulgaris and its variant, pemphigus vegetans; and the more superficially blistering pemphigus foliaceus and its variant, pemphigus erythematosus. All forms may occur at any age, but most present in middle age. The foliaceus form is especially apt to be associated with other autoimmune diseases, or it may be drug induced. Paraneoplastic pemphigus, a unique form of the disorder, is associated with numerous types of benign and malignant neoplasms (typically non-Hodgkin lymphoma). Pemphigus is characterized by an insidious onset of flaccid bullae, crusts, and erosions in crops or waves. In pemphigus vulgaris, lesions often appear first on the oral mucous membranes. These quickly become erosive. The scalp is another site of early involvement. Practitioners can rub a cotton swab or finger laterally on the surface of uninvolved skin which may cause easy separation of the epidermis (this is Nikolsky’s sign). The diagnosis is made with light microscopy and by direct and indirect immunofluorescence (IIF) microscopy. Autoantibodies to intercellular adhesion molecules can be detected with ELISA assays and have replaced the use of IIF in some centers. When the condition is severe, patients should be hospitalized at bed rest and given antibiotics/intravenous feedings. Anesthetic troches used before eating ease painful oral lesions. While pemphigus requires systemic therapy as early as possible, ironically, the main morbidity here is side effects from treatment. Initial therapy with systemic corticosteroids can consist of prednisone (60-80 mg daily). In most cases, a steroid sparing agent is added at the beginning at treatment (ex. azathioprine 100-200 mg daily, mycophenolate mofetil 1-1.5 twice daily). Treatment courses can be repeated in patients who do not achieve complete remission or relapse (ex. monthly IVIG at 2 g/kg intravenously over 3-4 days). In refractory cases, cyclophosphamide plus intravenous corticosteroids and plasmapheresis are also used. In patients who have a limited form of the disease, skin and mucous membrane lesions should be treated with topical corticosteroids. Complicating infection requires appropriate systemic and local antibiotic therapy. As far as complications are concerned, secondary infection can occur. This is a major cause of morbidity and mortality. Disturbances of fluid, electrolyte, and nutritional intake can occur as a result of painful oral ulcers. One in three patients will experience remission and, infection from S. aureus is the most frequent fatality cause.