Causative & risk factors
Pemphigus is an autoimmune disorder, caused by production of antibodies against one’s own body cells. The onset of pemphigus can be triggered by certain medications, X-rays, UV rays, hormones, pregnancy, stress, burns etc.
Even though pemphigus can affect people of any age group, those beyond middle age are more prone.
Clinical presentation
Pemphigus vulgaris presents as painful non-itching blisters in the oral cavity which later affect the skin. The armpits, groin and genitals are usually affected.
Pemphigus foliaceus presents with painless itching blisters, which usually begin on the head or face and eventually spread downwards.
The blisters may go on enlarging. They may burst open, leaving behind raw skin areas. Blisters in the oral cavity may cause hoarseness and difficulty in swallowing.
Investigations
After a symptomatic assessment, the doctor will elicit the ‘Nikolsky sign’. In this, light rubbing of the skin adjacent to a blister will cause peeling.
A skin biopsy may be performed and examined microscopically.
A blood test is done to detect desmoglein antibodies, which are suggestive of pemphigus.
Treatment
Pemphigus is treated with medications like corticosteroids and immunosuppressant drugs. Rituximab is a new drug being tried for treating pemphigus.
In case of secondary infections, antibiotics, antivirals or antifungal drugs may be prescribed. Intravenous immunoglobulin is given, if deemed necessary.
Certain patients with severe pemphigus may need to be hospitalized. Intravenous fluids and electrolytes are administered to these patients.
Plasmapheresis: This is a procedure in which the patient’s plasma is removed and then replaced with donated plasma. This helps to eliminate the antibodies responsible for pemphigus and thus helps in bringing down the symptoms.
Some patients with pemphigus make a complete recovery, whereas others may need medications for long periods.
