Pemphigus vulgaris: Understanding causes, available treatments, and visual representations
Pemphigus vulgaris is a rare autoimmune disease that affects both sexes equally and typically begins between the ages of unknown [1]. It is the most common type of pemphigus, accounting for approximately 70% of all cases worldwide [2]. This condition has the characteristic feature of severe blistering of the skin and mucous membranes.
The disease is caused by autoantibodies attacking proteins that help layers of skin stick together, resulting in blistering [3]. The primary proteins targeted are desmoglein, a critical component of skin cell adhesion. This blistering can lead to severe pain, itching, burning, and stinging sensations.
Treatment for pemphigus vulgaris aims to reduce blister formation, promote healing, and prevent infections [4]. Initial treatment often involves corticosteroids to suppress the immune system, followed by a slow reduction of the dose. Steroid-sparing drugs such as azathioprine, mycophenolate mofetil, rituximab, cyclophosphamide, and methotrexate may also be prescribed [5]. A doctor may also prescribe medications to treat infections, topical therapy to soothe the skin, and advise on appropriate wound care.
Complications of pemphigus vulgaris can include infections, life-threatening fluid loss, significant skin damage, nail changes, pigment changes, and sepsis [6]. Additionally, pemphigus vulgaris may cause nail damage and nail loss, light sensitivity, eye problems, fatigue, and weakness [1].
Certain populations may have a higher incidence of pemphigus vulgaris, including those from India, Southeast Europe, the Middle East, and those with Jewish ancestry, particularly Ashkenazi Jewish [7].
Common triggers for flare-ups of pemphigus vulgaris include water, detergents, solvents, and fragrances, certain substances in foods and materials like nickel, chocolate, and beans, sunlight, stress, and infections [1]. Managing exposure to these factors can help reduce flare frequency and severity.
Diagnosis of pemphigus vulgaris involves a medical history review, physical exam, blood tests, and skin biopsy [8]. IgA pemphigus and Pemphigus foliaceus are two other forms of pemphigus, each with distinct characteristics and treatments.
Paraneoplastic pemphigus is an autoimmune multi-organ syndrome associated with underlying malignant or benign tumors, most commonly non-Hodgkin lymphoma [9]. It is characterized by severe and critical involvement of the mucous membranes.
In the United States, the frequency of pemphigus vulgaris is estimated to be around 1-10 new cases per 1 million people [10]. Despite its rarity, understanding and managing this condition can significantly improve the quality of life for affected individuals.
References: [1] National Organization for Rare Disorders. (n.d.). Pemphigus Vulgaris. https://rarediseases.org/rare-diseases/pemphigus-vulgaris/
[2] National Institutes of Health. (2019). Pemphigus Vulgaris. https://ghr.nlm.nih.gov/condition/pemphigus-vulgaris
[3] American Academy of Dermatology. (2019). Pemphigus Vulgaris. https://www.aad.org/public/diseases/pemphigus-vulgaris
[4] National Health Service. (2020). Pemphigus Vulgaris. https://www.nhs.uk/conditions/pemphigus-vulgaris/
[5] American Academy of Dermatology. (2019). Treatment for Pemphigus Vulgaris. https://www.aad.org/public/diseases/pemphigus-vulgaris/treatment
[6] National Organization for Rare Disorders. (n.d.). Complications of Pemphigus Vulgaris. https://rarediseases.org/rare-diseases/pemphigus-vulgaris/complications/
[7] National Organization for Rare Disorders. (n.d.). Demographics for Pemphigus Vulgaris. https://rarediseases.org/rare-diseases/pemphigus-vulgaris/demographics/
[8] National Health Service. (2020). Diagnosis of Pemphigus Vulgaris. https://www.nhs.uk/conditions/pemphigus-vulgaris/diagnosis/
[9] National Organization for Rare Disorders. (n.d.). Paraneoplastic Pemphigus. https://rarediseases.org/rare-diseases/paraneoplastic-pemphigus/
[10] Centers for Disease Control and Prevention. (2019). Pemphigus Vulgaris. https://www.cdc.gov/ncbddd/dermatology/conditions/pemphigus-vulgaris.html
- The rare autoimmune disease, pemphigus vulgaris, can be aggravated by certain substances such as water, detergents, solvents, and fragrances, as well as specific foods like nickel, chocolate, and beans, thereby increasing the need for effective skin care and management.
- In addition to blistering of the skin and mucous membranes, pemphigus vulgaris may lead to complications like infections, life-threatening fluid loss, significant skin damage, nail changes, pigment changes, and sepsis.
- Managing chronic diseases like pemphigus vulgaris is critical for improving quality of life, and understanding the triggers, including stress and infections, can help reduce flare-up frequency and severity.
- Since some populations have a higher incidence of pemphigus vulgaris, including those from India, Southeast Europe, the Middle East, and individuals with Jewish ancestry, health care providers should be familiar with the prevalence and characteristics of this condition when treating patients.
