Pemphigus

Pemphigus is a set of autoimmune skin illnesses that can cause ulcers, blisters, or fluid-filled lumps on the skin and mucous membranes. Pemphigus is one of the most common autoimmune skin conditions, which often breaks open, hurts, and makes a person prone to subsequent infections.

Last Updated: February 21, 2024

 

About 1 to 5 out of every 1 million people in the world are diagnosed with pemphigus each year and the most common type of pemphigus is called pemphigus vulgaris. This accounts for 70% of diagnosed cases and this disease is characterized by blisters found in the oral cavity (most common), skin, and other mucous membranes.

 

People of all races, ages, and sexes can get pemphigus vulgaris and most people get it between 30 and 60 years old. 

 

Experts remain unsure what causes pemphigus, but they think the disease is affected by both genes and the environment.

 

Pemphigus is an autoimmune disorder. This means that the antibodies that are normally a part of the body’s defense system end up mistaking healthy cells as foreign bodies, attacking them, and forming blisters and sores on the skin.

 

Some medications, like penicillin, an antibiotic, piroxicam, a nonsteroidal anti-inflammatory drug used for rheumatoid arthritis, and blood pressure medications, can also cause the condition.

Most people with pemphigus vulgaris first notice sores on their mouth or genital mucous membranes. After a few weeks or months, blisters usually show up on the skin. However, in some cases, mucosal lesions may be the only sign.

 

Lesions on the skin look like soft, thin-walled blisters filled with clear fluid that can easily break open and cause itchy, painful erosions. Most of the time, they appear on the upper chest, back, head, and face. Erosion in the folds of the skin can turn into a granular, crusted, vegetative lesion (pemphigus vegetans). Some people have painful, red, and swollen skin around their nails.

 

Pemphigus vulgaris can cause extensive, life-threatening erosions if the disease isn't caught early enough. For example, severe complications of pemphigus include secondary bacterial, fungal, and viral infection. Fungal candida and herpes simplex are the most common secondary infections that happen.

In most cases, a blister biopsy is needed to figure out if someone has pemphigus vulgaris. But in some clinics, pemphigus vulgaris is usually diagnosed by taking a biopsy of the skin near a lesion. 

Laboratories will use direct immunofluorescence staining to look for immunoglobulin (Ig)G antibodies or complement on the surfaces of skin cells. Its presence confirms that a patient has pemphigus.

Most of the time, a blood test can find circulating antibodies (indirect immunofluorescence test). Enzyme-linked immunosorbent assays (ELISAs) can also be used to measure the amount of anti-dsg1 and anti-dsg3 antibodies in blood or saliva.

 

Treatment

The primary treatment for pemphigus is systemic corticosteroids. Corticosteroids are anti-inflammatory drugs that can be taken orally, injected, or applied topically to treat inflammation and its associated symptoms.

 

Another option is immunosuppressant drugs that diminish the immune system's ability to fight against its own healthy cells. Azathioprine or mycophenolate mofetil (MMF) are examples.

 

Monoclonal antibodies like rituximab are another modality doctors use since this drug specifically eliminates abnormal B cells

 

Intravenous immunoglobulin (IVIG) is the administration of healthy antibodies (proteins produced by your immune system to combat foreign substances) via a needle placed into a vein to lower the antibody levels.

 

In the case of secondary bacterial infections, doctors can prescribe antibiotics along with treating the cause of pemphigus vulgaris.

There are things that can be done to alleviate the symptoms.

  • Crunchy, acidic, or spicy foods should be avoided since they can exacerbate blisters in the mouth and throat
  • Taking care of the blisters in the manner prescribed by the doctor
  • To keep UV rays from hurting the skin, stay out of the sun, cover the skin with sunscreen, or wear protective clothing
  • To avoid skin irritation, use fragrance-free soaps and lotions formulated for sensitive skin

 

References

Ingold CJ, Khan MAB. Pemphigus Vulgaris. [Updated 2022 Aug 8]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK560860/

Oakley, A., Shakeel, M (2022). Pemphigus vulgaris. Retrieved January 14, 2023, from https://dermnetnz.org/topics/ pemphigus-vulgaris

Cleveland Clinic (2023). Pemphigus. Retrieved January 14, 2023, from https://my.clevelandclinic.org/health/diseases/21130-pemphigus

Last Updated: February 21, 2024