Pemphigus Vulgaris

Pemphigus Vulgaris
Pemphigus is a group of rare autoimmune blistering disorders of the skin in which blisters and sores occur in skin and mucous membrane. It is a disease of immune system. The immune system produces antibodies which normally attack hostile viruses and bacteria in the blood in an effort to keep us healthy. In an affected individual however, the antibodies erroneously perceive the skin and/or mucous membrane tissue as foreign and attacks them. This produces burn-like lesions that will not heal. In some cases, these sores can cover a significant area of the skin. The disease can give a horrifying image and if not managed properly may lead to death. There is no cure for this disease. Steroids are only hope and give dramatic relief.
There are three main categories of Pemphigus.Paraneoplastic Pemphigus, Pemphigus vulgaris and Pemphigus foliaceusPemphigus vulgarisIt is most frequently diagnosed form of pemphigus. Sores and blisters almost always start in the mouth. Large bullae occur on skin. Those in the oral cavity soon rupture leaving a ragged slough over a shallow ulcer. It has a high rate of mortality.

There is a research which shows that there is a genetic predisposition to Pemphigus Vulgaris, based primarily on the observation that this disease appears to occur more frequently than normal in Askenazi Jews.

Microscopic Features: Characteristic features under microscope are “Intraepithelial clefting” and “tombstoning”.


 Pemphigus Vulgaris
Pemphigus foliaceusWith pemphigus foliaceus, blisters and sores do not occur in the mouth. Crusted sores or fragile blisters usually first appear on the face and scalp and later involve the chest and back.The blisters are superficial and often itchy, but are not usually as painful as in pemphigus vulgaris. In PF disfiguring skin lesions can occur but the mortality rate from the disease is much lower than in PV  Pemphigus Vulgaris
Paraneoplastic pemphigusThis is the most serious form of pemphigus, which occurs most often in someone who has already been diagnosed with a Cancer.

 Paraneoplastic Pemphigus

 Treatment

Prednisone is the most important medication for these patients and those not put on steroids may rapidly deteriorate and die. Dose of prednisone has to be according to the need of individual patient. Other drugs used include Azathioprine, CyclophosphamideMethotrexate, Dapsone, GoldTetracyclineMinocycline.

Diet high in protein, low in carbohydrates, low in salt, low in fat is beneficial. In extensive lesions fluid loss may have to be managed. Mouth lesions may hamper nutrition of the patient which also has to be taken care of. Blood calcium and potassium levels may be disturbed. Fungal infections have also to be managed in such patients.

 Skin Diseases
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