Pemphigus vulgaris (PV) involves the formation of blisters on the skin and mucous membranes. Though rare, this disease is quite significant since it is potentially life-threatening, with a high mortality rate of 5-15%. Blister formation in the skin and mucous membranes is a hallmark of this disease, caused due to the presence of circulating antibodies directed against the keratinocyte cell surfaces in the skin, thereby causing a loss of cell-to-cell adhesion and a breach in the skin epidermis. Blisters may appear on normal or inflamed skin; are of varying sizes; are fragile and rupture easily; are painful and heal slowly, usually without scarring. Oral cavity involvement is the commonest presentation; other mucous membranes which may be involved include the conjunctiva, esophagus, labia, vagina, cervix, vulva, penis, urethra, nasal mucosa, and anus.
Skin biopsy from the edge of a blister can help in accurate diagnosis. There are two tests in routine use: direct immunoflourescence (DIF) on normal-appearing skin surrounding the blister or plucked hair sheaths; and indirect immunoflourescence (IDIF) using the patient’s serum. Antibody titers correlate well with disease activity and can be detected using ELISA tests. Antidesmoglein 3 antibodies indicate mucosal involvement; while antidesmoglein 1 levels help predict the course of the disease. Reversion of DIF test to negative can be used as an indicator of remission and for monitoring while tapering medicines.
Corticosteroids are used as the mainstay of PV treatment to reduce and stop the inflammation process; while immune suppressing medicines are sometimes used early on in the course of the disease as steroid-sparing medication. Susceptibility to infection, as well as fluid and electrolyte imbalance contributes to fatalities, which are more common in the first 5 years of the disease. 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, with elderly patients and patients with extensive disease having a more serious prognosis. The long term use of steroids and immune suppressants also contributes to the overall morbidity and mortality. Steroid-sparing drugs include rituximab, sulfasalazine, pentoxyphylline, methotrexate and dapsone. Intravenous immunoglobin therapy and plasmapheresis have been used with some degree of success in refractory patients.
Ayurvedic herbal medicines have a significant role to play in the overall long term treatment and management of PV, considering the high mortality of this disease as well as the contributing toxicity of steroids and immune suppressants drugs. As with all autoimmune disorders, the treatment protocol for this disease 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.
The Ayurvedic treatment for strengthening the integrity of the skin and mucous membranes involves the use of medicines which act specifically on the skin and mucous membranes as well as on blood vessels. Immune modulating herbs which act specifically on skin and mucous membranes are very useful in this scenario. Additional medicines are also required to help in healing of ulcers, and for the prevention of secondary infection in the sores.
Ayurvedic detoxification protocol 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 are highly potent and have the potential to 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.
Ayurvedic herbal medicines may need to be given for periods ranging from about 6 to 10 months, with more than 80 % patients achieving full remission. Recurrence can be prevented by gradual tapering of medicines, as well as suitable modifications in diet and lifestyle. Aggravating factors like stress and certain medications also need to be avoided. Ayurvedic herbal treatment can thus bring about significant improvement in PV and considerably reduce the mortality and morbidity due to this condition.