Written by: Lucky Annem
Medically reviewed by: Dr. Lohi Pratti
Pemphigus is a rare autoimmune disease characterized by blistering of the skin and mucous membranes. It is a group of autoimmune bullous diseases that causes the immune system to attack healthy skin cells, leading to blister formation. Being an autoimmune condition, pemphigus is brought about by autoantibodies to an epidermal antigen. The most common types of pemphigus are pemphigus vulgaris (PV) and pemphigus foliaceus (PF). PV is the most common and severe form, characterized by the formation of intraepidermal blisters, whereas PF is characterized by superficial blisters in the upper layer of the skin.
Pemphigus can affect people of any age, gender, and ethnicity, but it is most commonly seen in individuals aged 50-60 years. Women are affected more frequently than men. It is more commonly seen in people of Middle Eastern, Jewish, and Mediterranean descent, but it can occur in people of any ethnic background. Additionally, individuals with certain genetic profiles may have an increased risk of developing pemphigus. People with a family history of autoimmune diseases, particularly pemphigus, may also have an increased risk of developing the condition.
The symptoms of pemphigus can vary depending on the type and severity of the disease. In general, the symptoms of pemphigus include blisters or sores on the skin and mucous membranes that can be painful, itchy or both. Itching may act as the prime clinical sign of this disease. These blisters can occur anywhere on the body, but are most commonly found on the face, scalp, chest, and back. They can also affect the mouth, throat, and genitals. As the blisters rupture, they leave behind painful ulcers that can slowly heal. In some cases, pemphigus can also cause fever, fatigue, and body aches. Rare cases of hand and foot involvement in pemphigus are thought to be poor prognostic indicators. It is important to seek medical attention if you experience any of these symptoms, as early diagnosis and treatment can help prevent complications and improve outcomes.
The treatment for pemphigus is focused on reducing inflammation and managing symptoms. The primary treatment is usually corticosteroids, which work by suppressing the immune system and reducing inflammation. In more severe cases, immunosuppressive drugs may also be used. A study found that with the active and widespread use of corticosteroids, the death rate from pemphigus which was previously as high as 90%, dropped a lot. Other treatments that may be used include rituximab, which is a monoclonal antibody that targets B cells, and intravenous immunoglobulin, which is a concentrated solution of antibodies derived from blood donors. Additionally, doctors may recommend topical treatments for skin lesions, such as corticosteroid creams or ointments. Individuals with pemphigus need to receive ongoing medical care and monitoring to manage their symptoms and adjust their treatment plan as necessary.
Living with pemphigus can be challenging both physically and emotionally. The physical symptoms can cause discomfort, pain, and limitations in daily activities such as eating, talking, and walking. The painful blisters and sores may lead to weight loss, malnutrition, and dehydration. Patients may also experience fatigue and weakness due to the stress on their immune systems. Additionally, the emotional toll of pemphigus should not be underestimated. Patients may experience anxiety, depression, and social isolation due to the appearance of their skin, and the pain and discomfort associated with the condition.
A personal testimony from a patient with pemphigus:
"My name is Sarah and I was diagnosed with pemphigus vulgaris about five years ago. At first, it was very scary because I had never heard of it before and didn't know what to expect. The blisters and sores on my skin and in my mouth were painful and made it difficult to eat and talk. I also experienced hair loss due to the medication.
Over time, I learned to manage my symptoms through a combination of medication and lifestyle changes. I now follow a strict diet that avoids certain trigger foods and take medication to suppress my immune system. I also take extra care with my skin and oral hygiene to prevent infections.
Living with pemphigus can be challenging at times, but I have learned to adapt and find joy in the things I can still do. I have a supportive network of family and friends who have helped me through the ups and downs. Although it can be tough, I remain hopeful that with continued research and treatment advancements, a cure for pemphigus will one day be found."
Coping with the chronic nature of the disease, the unpredictability of flare-ups, and the impact on one's personal and professional life can also be challenging. Therefore, a supportive network of family, friends, and healthcare providers can be vital in helping patients navigate the challenges of living with pemphigus. Pemphigus is a rare autoimmune disease that affects the skin and mucous membranes. It can be life-threatening if left untreated and can have a significant impact on a patient's quality of life. Early diagnosis and treatment are essential to manage the symptoms and prevent complications. More research is needed to better understand the causes of pemphigus and to develop more effective treatments with fewer side effects.
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References:
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Lane, Anna. “Patient Stories | IPPF.” Pemphigus.org, http://www.pemphigus.org/patient-stories/. Accessed 6 May 2023.
Lowe, Siri. “Pemphigus vulgaris.” PubMed, 1 December 2007, https://pubmed.ncbi.nlm.nih.gov/18048542/. Accessed 6 May 2023.
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“Pemphigus - Symptoms and causes.” Mayo Clinic, 27 September 2022, https://www.mayoclinic.org/diseases-conditions/pemphigus/symptoms-causes/syc-20350404. Accessed 6 May 2023.
“Pemphigus: Types, Symptoms, Causes & Treatment.” Cleveland Clinic, 18 May 2022, https://my.clevelandclinic.org/health/diseases/21130-pemphigus. Accessed 6 May 2023.
“Pemphigus Vulgaris.” Johns Hopkins Medicine, http://www.hopkinsmedicine.org/health/conditions-and-diseases/pemphigus-vulgaris. Accessed 6 May 2023.
“Treatment of pemphigus: an Indian perspective.” PubMed, https://pubmed.ncbi.nlm.nih.gov/25035350/. Accessed 6 May 2023.
Tsuruta, Daisuke, et al. “Diagnosis and Treatment of Pemphigus.” Medscape, http://www.medscape.com/viewarticle/769843. Accessed 6 May 2023.
“What is Pemphigus? Symptoms & Causes | NIAMS.” National Institute of Arthritis and Musculoskeletal and Skin Diseases, 1 January 2021, http://www.niams.nih.gov/health-topics/pemphigus. Accessed 6 May 2023.
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