Pemphigus is a rare and serious autoimmune disease that mainly affects the skin and mucous membranes (such as the mouth, pharynx, esophagus, conjunctiva, and genital area). The disease occurs when the immune system produces antibodies that attack the proteins responsible for binding skin cells together (desmogleins). This aggression leads to the separation of the skin layers, forming fragile blisters and painful ulcers.
Pemphigus is caused by an autoimmune reaction in which the body attacks the proteins (desmogleins) that bind skin cells, being influenced by genetic factors, drugs, viral infections, or exposure to toxic substances.
It manifests itself by the appearance of fragile blisters and painful ulcers on the skin and mucous membranes, accompanied by tenderness, difficulty swallowing, and an increased risk of infection.
Treatment includes corticosteroids, immunosuppressants, biological therapy with rituximab, plasmapheresis, and local care to control inflammation, prevent infections, and reduce immune system activity.
H2: What is pemphigus?
Pemphigus is a rare autoimmune disease that affects the skin and mucous membranes, characterized by the appearance of painful blisters and ulcers. It occurs when the immune system attacks the proteins that hold skin cells together, leading to the separation of skin layers and the formation of lesions. It is a chronic condition that requires long-term treatment to prevent relapses and complications.
H2: How does pemphigus manifest itself in the body?
Pemphigus manifests itself in soft blisters that break easily and leave painful ulcers on the skin and mucous membranes, especially in the mouth, esophagus, nose or genitals. Ulcerations cause pain, difficulty eating or swallowing, and the affected areas become sensitive and exposed to secondary infections. In severe forms, the disease can affect the general condition of the body, causing fever, fatigue and dehydration.
H2: Main types of pemphigus
Pemphigus includes several subtypes, each with distinct clinical features and varying degrees of severity. These forms affect the skin and/or mucous membranes in specific ways.
H3: Pemphigus vulgaris
Pemphigus vulgaris is the most common form, characterized by blisters and ulcerations that initially appear in the oral cavity and may spread to the skin. The lesions are painful and often begin as small blisters that rupture quickly, leaving open sores.
H3: Pemphigus foliaceus
This form of pemphigus foliaceus affects only the skin, without mucosal involvement, and is manifested by superficial blisters that usually appear on the scalp, face, and trunk. The blisters rupture easily, leaving behind scaly, burn-like areas.
H3: Bullous Pemphigus and Bullous Pemphigoid
Although similar in name, these are different conditions. Bullous pemphigus is a rare variant of pemphigus, characterized by large, fluid-filled blisters, while bullous pemphigoid is a distinct autoimmune condition, more common in the elderly, with firmer, less painful blisters.
Bullous Pemphigus Treatment: Treatment for Bullous Pemphigus usually includes medications such as corticosteroids and immunotherapy to reduce inflammation and prevent complications.
Bullous Pemphigoid Treatment: Bullous Pemphigoid treatment focuses on the administration of corticosteroids and immunosuppressants to reduce autoimmune reactions.
H3: Paraneoplastic Pemphigus
This form occurs in association with certain types of cancer (for example, lymphomas or leukemias). It affects both the skin and mucous membranes, with various manifestations, including painful skin lesions, mouth ulcers and sometimes lung inflammation. It is a severe and difficult-to-treat form.
Paraneoplastic pemphigus: Paraneoplastic pemphigus is associated with certain types of cancer and requires specific treatment to manage both the autoimmune condition and the tumor.
H2: Causes and risk factors in pemphigus
Pemphigus is caused by an autoimmune reaction in which the immune system attacks the proteins (desmogleins) that hold skin cells together, causing them to separate and form blisters. The main risk factors include:
▪️Genetic predisposition: People with a family history of autoimmune diseases are at higher risk.
▪️Medications: Some medications, such as penicillamine, captopril or antibiotics, can trigger the disease.
▪️Environmental factors: Exposure to toxic substances or UV radiation can influence the onset of the disease.
▪️Associated conditions: Coexisting autoimmune diseases or certain types of cancer (in the case of paraneoplastic pemphigus).
H2: Symptoms of pemphigus
Pemphigus manifests itself by:
▪️Fragile blisters: They appear on the skin and mucous membranes (especially in the mouth), they break easily and leave painful ulcers.
▪️Pain and functional difficulties: Oral ulcerations make it difficult to eat, drink and speak.
▪️Skin sensitivity: The affected skin becomes irritated, sensitive and prone to infections.
▪️Fever and general malaise: Can occur in severe cases, especially if the ulcers become infected.
▪️Chronic lesions: Slow healing can lead to long-term discomfort and dehydration in extensive forms.
H2: How is pemphigus diagnosed?
The diagnosis of pemphigus is based on a combination of clinical examination, laboratory tests, and additional investigations to confirm the presence of the disease.
H3: Medical history and clinical examination
The doctor begins by evaluating the patient's medical history, paying attention to symptoms, onset of the disease, and any precipitating factors (such as medications or associated diseases). Physical examination reveals the presence of fragile blisters, ulcerations, and the characteristic appearance of the lesions on the skin and mucous membranes.
H3: Skin biopsy and immunological tests
Skin biopsy: A sample of skin is examined microscopically to identify the separation of skin cells, characteristic of pemphigus.
Direct immunofluorescence: Identifies specific autoimmune antibodies in skin tissues.
Serological tests: Indirect immunofluorescence and ELISA are used to detect anti-desmoglein antibodies circulating in the blood.
H3: Further evaluation
In complex or atypical cases, further investigations may be performed to rule out other conditions or complications:
Blood tests: To assess inflammation, organ function, or the presence of secondary infections.
Endoscopy: If there are symptoms in the esophagus or other internal areas affected by pemphigus.
Tests for associated diseases: In the case of paraneoplastic pemphigus, malignancies or other associated autoimmune diseases are looked for.
H2: Treatment options for pemphigus
Treatment of pemphigus is complex and aims to reduce inflammation, control symptoms and prevent complications.
H3: Immunosuppressive drugs and corticosteroids
Corticosteroids: Prednisone is used to quickly control acute inflammation and symptoms. In severe cases, it is given intravenously.
Immunosuppressants: Immunosuppressive drugs help reduce the activity of the immune system and prevent relapses.
H3: Additional therapies and local care
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This integrative protocol is designed to support the body in the process of cleansing toxins, strengthening the immune system and restoring blood balance, having a positive impact on overall health and the management of autoimmune diseases such as pemphigus.
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Adjuvant therapy Curcumin & Reveratrol infusions
Ozone Therapy - Autohemotherapy
Ozone autohemotherapy is a therapeutic method used in the treatment of autoimmune diseases, including pemphigus, due to its immunomodulatory and anti-inflammatory effects. The procedure consists of collecting a quantity of blood from the patient, mixing it with a certain concentration of ozone and reintroducing it into the body through an intravenous infusion.
This technique helps reduce oxidative stress, stimulate the immune system and improve blood circulation, thus contributing to the improvement of pemphigus symptoms and a better quality of life for patients. This method is used in various autoimmune diseases, including pemphigus. Learn more about Autohemotherapy - CLICK HERE. - https://www.quantica720.ro/service/autohemoterapie
H3: Diet and Lifestyle
Adopting a balanced and healthy diet plays a fundamental role in managing pemphigus and improving overall health. Integrative nutrition is a holistic approach to diet, which not only supports healing, but also contributes to the prevention of inflammation and other symptoms of autoimmune diseases, such as pemphigus.
Pemphigus vulgaris nutrition: An anti-inflammatory diet is essential for people with Pemphigus vulgaris, contributing to the improvement of symptoms.
Gut microbiome link
The principles of integrative nutrition are based on the understanding that the health of the body is closely linked to what we consume, and that food is not only a source of energy, but also a tool for regulating physiological functions.
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H2: Can pemphigus be cured completely?
Pemphigus is a chronic, autoimmune disease that cannot be completely cured, but can be effectively controlled with appropriate treatments. The goal of treatment is to reduce inflammation, prevent relapses, and improve symptoms. In some cases, patients may experience long periods of remission, in which symptoms are minimal or absent, but since it is an autoimmune condition, continued control of the disease with medications and supportive therapies remains necessary in the long term.
H2: Complications of pemphigus
Pemphigus can lead to a number of serious complications, especially in severe or untreated forms, including:
▪️Infections of the skin and mucous membranes: Open lesions are vulnerable to bacterial, viral, or fungal infections, which can complicate the healing process.
▪️Dehydration: Fluid loss through severe ulcerations can lead to dehydration, especially when they affect large areas of skin.
▪️Dietary and respiratory difficulties: Ulcers in the mouth or esophagus can make swallowing difficult, and lesions in the airways can affect breathing.
▪️Systemic risks: In severe cases or in the absence of treatment, pemphigus can lead to general immune disorders, affecting various organs and body systems.
Prompt treatment and appropriate management of the disease can significantly reduce the risk of these complications and improve the quality of life of patients.
H2: Conclusion
Pemphigus is a rare and chronic autoimmune disease that affects the skin and mucous membranes, with a significant impact on the patient's quality of life. Although it cannot be completely cured, with appropriate treatment and constant monitoring, symptoms can be effectively controlled and periods of remission can be prolonged. It is essential that patients follow a personalized treatment plan, which may include immunosuppressive drugs, additional therapies and local care.
Also, a healthy diet, with an emphasis on anti-inflammatory nutrients and a balanced lifestyle, play a crucial role in managing the disease. Complications of pemphigus, such as infections or swallowing difficulties, can be prevented by preventive treatments and appropriate care. Overall, with early diagnosis and integrated management, patients can live active and healthy lives, minimizing the impact of the disease on their health.
Bibliography
Mutasim, D. F., Pelc, N. J., & Anhalt, G. J. (1993). Paraneoplastic pemphigus, pemphigus vulgaris, and pemphigus foliaceus. Clinics in dermatology, 11(1), 113-117.
Bystryn, J.C., & Rudolph, J.L. (2005). Pemphigus. The Lancet, 366(9479), 61-73.
Articles online
https://www.niams.nih.gov/health-topics/pemphigus
https://my.clevelandclinic.org/health/diseases/21130-pemphigus
