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.
