Pemphigus is a group of skin disease that usually causes blisters, sores or pus-filled bumps on the skin or mucous membranes, such as in the mouth, soft linings of the eyes, nose, throat, or on the genitals. It is an auto-immune disorder which can spread over large areas of the body and have a high risk of infection. Since it causes blistering skin, it is often confused with other skin conditions like Bullous pemphigoid, Lupus erythematosus, or Hailey-Hailey disease. Although pemphigus isn’t contagious, it can be a life-long condition that can be managed with proper medical treatment.
Also Read: Morphea: Causes, Symptoms And Treatment
Types
Pemphigus can be of different types based on where or why the blisters develop. These include:
Pemphigus Vulgaris: Blisters usually affect the mouth, skin and in other mucous membranes. These lesions mainly develop in the deep layers of the skin and are often painful and slow to heal.
Pemphigus Vegetans: Lesions that occur are thicker and wart-like and usually form in the areas of the body with skin folds such as the groin and armpit.
Drug-induced Pemphigus: Blisters may develop due to the use of medicines like Penicillin or piroxicam.
Pemphigus Erythematosus (Senear-Usher syndrome): This type involves blisters that mainly develop on the upper back, chest, cheeks, and scalp and the lesions formed are usually red and crusty.
Pemphigus Foliaceus: In this type, the blisters develop on the scalp and often on the face, neck, and back. Small blisters may easily break to form crusted lesions that spread to cover large areas of the skin.
Endemic Pemphigus (fogo selvagem): This form of pemphigus often affects multiple family members.
Paraneoplastic Pemphigus: A rare form of pemphigus that mainly affects people with cancer.
Causes
Although pemphigus is an autoimmune disorder, the exact cause of it is still unknown. In general, the body’s immune system produces antibodies to combat harmful invaders, such as viruses and bacteria. But in case of pemphigus, the body produces antibodies that attacks it’s own cells and mucous membranes. In rare case scenarios, pemphigus is triggered by the use of angiotensin-converting enzyme inhibitors, penicillamine or other drugs.
Risk Factors
Certain causative factors that increase the risk of pemphigus include:
Age: It is more commonly noticed in older or middle-aged people than their younger counterpart
Ethnicity: People of Middle Eastern or Jewish descent are at a higher risk of developing this skin condition
Symptoms
The characteristic symptom of Pemphigus is blisters on the skin and mucous membranes. These blisters can rupture easily, leaving open sores, which may ooze and become infected. This blistering skin is also quite painful, have a burning effect and itch continuously.
Complications
If the condition is not treated on time, it can cause several complications including:
- Skin infection
- An infection that spreads to the bloodstream (sepsis)
- Malnutrition, (painful mouth sores make it difficult to eat or drink)
- Medication side effects
- Death
Diagnosis And Treatment
In case you notice any of the above mentioned signs and symptoms, do consult a doctor at the earliest to avoid further complications. The doctor usually does a thorough physical check-up to look for blisters or skin that easily peels, acknowledges the patient’s medical history and conducts a few diagnostics which include:
- Blood tests to identify the antibody that has caused pemphigus
- Skin biopsy to rule out cancerous condition
- Endoscopy to detect sores in the throat
Also Read: Gastroscopy: Procedure, Risks And Results
Treatment
Treatment should begin as early as possible in order to stop the spread of blisters as soon as possible. The treatment options for pemphigus includes:
- Stopping the use of the medication in case of drug-induced
- Corticosteroids
- Steroid-sparing immunosuppressant medications
- Plasmapheresis
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