Pemphigus vulgaris (PV) is a rare autoimmune disease involving the formation of blisters on the skin and mucous membranes. This disease is quite significant since it is potentially life-threatening, with a high mortality rate of 5-15%, even though affecting only the skin and mucous membranes. Circulating antibodies are directed against the keratinocyte cell surfaces in the skin; this causes a loss of cell-to-cell adhesion, resulting in a breach of the skin epidermis, thereby causing blisters. These blisters are of varying size, and may appear on normal or inflamed skin. The blisters are fragile and rupture easily; these are painful and heal slowly, usually without scarring. Almost all patients present with oral cavity involvement; other mucous membranes which may be involved include the conjunctiva, esophagus, labia, vagina, cervix, vulva, penis, urethra, nasal mucosa, and anus.
Diagnosis is usually made by skin biopsy from the edge of a blister; direct immunoflourescence (DIF) on normal-appearing skin surrounding the blister or plucked hair sheaths; and indirect immunoflourescence (IDIF) using the patient’s serum. ELISA tests can detect the presence of antibodies and these titers correlate well with disease activity. While antidesmoglein 3 antibodies are present in patients having only mucosal involvement, the course of the disease correlates well to antidesmoglein 1 antibody levels. Reversion of DIF test to negative can be used as an indicator of remission and for monitoring while tapering medicines.
Treatment of PV is mainly with corticosteroids to reduce and stop the inflammation process. Immune suppressing medicines are sometimes used early on in the course of the disease as steroid-sparing medication. Fatalities are more common in the first 5 years of the disease, and are related to susceptibility to infection, as well as fluid and electrolyte imbalance. Morbidity and mortality is related to the severity and extent of the disease, the dosage of steroids required to induce remission, as well as the presence of co-morbidities. Elderly patients and patients with extensive disease have a more serious prognosis. The long term use of steroids and immune suppressants also contributes to the overall morbidity and mortality. Rituximab, sulfasalazine, pentoxyphylline, methotrexate and dapsone have been used as steroid-sparing drugs. Intravenous immunoglobin therapy and plasmapheresis have been used with some degree of success in refractory patients.
Because of the high mortality of this disease as well as the contributing toxicity of steroids and immune suppressants drugs, Ayurvedic herbal medicines have a significant role to play in the overall long term treatment and management of PV. This being an autoimmune disorder, the treatment protocol includes a multipronged approach of detoxification, proper nutrition, rejuvenation of body systems, immune modulation, as well as specific treatment for the actual systems or organs affected.
Special attention is focused on strengthening the integrity of the skin and mucous membranes. This involves the use of medicines which act specifically on the skin and mucous membranes as well as on blood vessels. Herbal medicines which have immune modulating properties as well as act specifically on skin and mucous membranes are very useful in this scenario. Medicines also need to be given to help in healing of ulcers, and for the prevention of secondary infection in the sores.
Detoxification for each patient needs to be tailor-made according to the severity and chronicity of PV lesions. While some patients may require just a few additional medicines to boost kidney and liver function, yet others may require an elaborate detoxification plan for induced emesis, induced purgation, and blood-letting. Known in Ayurveda as Panch-karma, these procedures may be used as standalone or as combination-procedures. These detoxification procedures may provide rapid remission of PV symptoms; however, patients need to be selected carefully, since most affected with PV are old or have concurrent comorbid conditions.
Depending upon the severity of the condition as well as the response of patients to treatment, Ayurvedic herbal medicines may need to be given for periods ranging from about 6 to 10 months. With regular treatment, most patients affected with PV respond well to Ayurvedic herbal treatment and more than 80 % achieve full remission. Gradual tapering of medicines, as well as suitable modifications in diet and lifestyle, can help prevent recurrence of the condition. Aggravating factors like stress and certain medications also need to be avoided. A judicious utilization of Ayurvedic herbal treatment can thus bring about significant improvement in PV and considerably minimize the mortality due to this condition.
The writer, Dr A A Mundewadi is available as an Ayurvedic Consultant at www.mundewadiayurvedicclinic.com and www.ayurvedaphysician.com