When autoimmune diseases are mentioned, people typically think of type 1 diabetes or celiac disease. They rarely think of pemphigus – a far lesser known condition which causes the body’s immune system to attack its own skin.
Pemphigus has been spotlighted recently by Dr Kalyani Bhola, a dermatologist at Alchemist Hospital. Bhola spoke of pemphigus in a public lecture delivered in Panchkula to raise awareness of the condition.
Pemphigus is largely unknown by the general population due to its relative rarity. Of the few studies conducted within India it was found that the prevalence among dermatology outpatients was 0.09 to 1.8 percent based on a clinic-based questionnaire survey conducted in the Thrissur district of Kerala.
However, two things have been noted from these studies that show concerning trends among the Indian population. Firstly, the incidence of the disease is higher in India than in western countries. This is not uncommon in a genetic disorder, as certain populations may harbour genes that make them more prone to the condition. An example of this is the genetic predisposition of the Indian population towards developing diabetes.
“Pemphigus is largely unknown by the general population due to its relative rarity…however…the incidence of the disease is higher in India than in western countries”
It was also found that, uniquely among the Indian population, the disease tends to occur earlier in life. For the rest of the world the standard age of onset is around forty to sixty years of age. In India more than half of the patients were diagnosed before the age of forty, potentially indicating that the genetic susceptibility extends to an earlier age of onset.
Dr Bhola highlights that an autoimmune disease can affect any organ within the body. The most affected location tends to be the skin.
In the case of pemphigus, symptoms begin with blisters. They start in the mouth and, within a few months, also begin appearing on the external skin. The blisters commonly affect the scalp, face, neck and upper parts of the chest and back, though can occur on the genitals as well as in the throat.
The blisters tend to occur in sporadic episodes and can sometimes become severe enough that, upon bursting, large areas of exposed skin are left that are prone to infection. It is in this aspect that pemphigus can prove fatal.
While the disease cannot be cured, it can be managed. Flare-ups of the conditions can be monitored to ensure medications such as corticosteroids are provided in advance of symptoms worsening.