Bullous pemphigoid is an important skin disease that causes non-deep wounds that crust over with the bursting and opening of fluid-filled blisters on the body surface over time. The disease does not have a different name used among the society. Bullous diseases are in the group of autoimmune diseases. Local or widespread bullous (fluid-filled bubble) lesions are seen accompanied by itching. In 20% of affected patients, bullae do not appear at all. Bullous pemphigoid disease, especially accompanied by bullae, adversely affects the quality of life of the person. Bullous pemphigoid disease can be seen in any age group, but it affects the elderly population more. The disease is rarely seen in children. The incidence of the disease does not differ according to gender, it is equally distributed in men and women.
What is Bullous Pemphigoid?
Bullous pemphigoid is the most common in the category of autoimmune skin diseases. Its incidence increases with advancing age and is more common in individuals over 60 years of age. The incidence of the disease is equal in men and women. However, there are studies in which women are affected more by the disease depending on racial characteristics, HLA genotypes and environmental factors. Although the disease is rare in children, it affects girls more frequently. If bullous pemphigoid is not treated, it can turn into a chronic disease that negatively affects the quality of life of the individual. Bullous pemphigoid disease is not contagious. The patient's wounds are not transmitted by contact, blood or other ways. Gestational pemphigoid (Pemphigoid Gestationes) is a special type of bullous pemphigoid disease seen during pregnancy. The disease occurs in the second or third trimester of pregnancy, sometimes after childbirth. The redness starts in the navel and abdomen and then spreads to the whole body. Bleeding after redness is followed by the formation of bullae. While the bullae heal, they do not leave a scar (scar), but spots on the skin and, rarely, small superficial keratin cysts (milia) occur.
What Causes Bullous Pemphigoid Disease?
The body's immune system mechanism's own skin Bullous pemphigoid disease occurs as a result of the severe reactions to the skin layer. As a result of these abnormal reactions, the autoa Although the cause of antibodies is not known exactly, some factors trigger the disease. treatment
- Rheumatoid Arthritis (joint rheumatism)
- Hashimoto's Thyroiditis (the most common thyroid gland disease)
- Dermatomyositis (with skin rash and muscle weakness) inflammatory disease)
- Lupus Erythematosus (rheumatic disease causing multiple organ involvement)
- Autoimmune Thrombocytopenia (low platelet count in the blood accompanied by autoimmune diseases)
- Inflammatory Bowel Diseases (inflammatory bowel diseases affecting the digestive system)
- Multiple Sclerosis (nervous system disease)
- Amyotrophic Lateral Sclerosis (nervous system disease)
What are the Symptoms of Bullous Pemphigoid Disease?
The most common symptoms of bullous pemphigoid disease are itching and bulla formation. The inside of the bullae is usually filled with clear fluid, but can sometimes be hemorrhagic (bloody). The size and density of the lesions vary. Lesions remain in the body for long periods of time, such as weeks or months. The most common areas are surfaces such as armpits, groin, elbow bends. Physical symptoms vary according to the subgroups of bullous pemphigoid disease. Mucosal lesions occur in 10-30% of patients with classic bullous pemphigoid. While mucosal involvement mostly covers the oral (mouth) mucosa, the eye, nose, pharynx (pharynx), esophagus (esophagus) and genital mucosa are rarely affected. As in many autoimmune diseases, the evaluation of bullous pemphigoid pathology is the most important step in the diagnosis of the disease. Bullous pemphigoid used in the diagnosis of methods are:
- Physical examination
- Histopathological tests
- Direct and indirect immunofluorescence tests
- Various serous tests
- Skin Immunofluorescence examination from biopsy is very important in the diagnosis and typing of pemgphioid. count
- Blood electrolytes
- Erythrocyte sedimentation rate
- Gamma-Glutamyl Transferase (GGT)
- C-Reactive Protein test (CRP)
- Fasting Blood Glucose test,
- Alkaline Phosphatase, Bilirubin, Creatinine test
- Serum Albumin test
- Pre-treatment pregnancy test if necessary
- Performing echocardiography (examination of the heart by ultrasonography).
- Performing bone densitometry (bone measurement)
- Performing eye examination (eye pressure and cataract control)
- Hepatitis B, Hepatitis C, and HIV serology
- Tiopurine Methyltransferase test
- Glucose 6-Phosphate Dehydrogenase test
- Exclusion of evidence of serum IgA deficiency
- Taking samples for bacterial investigations
How is the Treatment of Bullous Pemphigoid Disease?
Bullous pemphigoid doctors are experienced dermatologists. In the presence of the patient's general condition, clinical picture and other accompanying diseases, dermatologists, family physicians, neurologists, geriatricians, geriatric nurses, psychologists and physiotherapists take part in patient treatment as a team. At the same time, family members also play an active role in the care of the patient. Due to the recurrent nature of the disease and its chronicity, important steps play a role in the follow-up and treatment. These important steps are as follows:
- Confirmation of the diagnosis of bullous pemphigoid by evaluating signs and symptoms
- Confirming risk factors and other accompanying diseases. Investigation of the pain
- Determination of bullous pemphigoid type and severity
- Evaluation of the course of the disease and general condition according to age
- Review of individual and most appropriate treatment options
- Immunosuppressive agents
- Anthyrheumatic drugs
- Intravenous immunoglobulin (intravenous antibody) therapy
- Plasmapheresis (plasma exchange therapy).
- The drugs should be used regularly to prevent the disease from recurring.
- Long-term exposure to the sun should be avoided.
- Sunscreen with a high protective factor should be used.
- Appropriate clothing with a high cotton content and not tight on the body should be worn.
- Warm water to prevent skin irritation shower with. Very hot or very cold water should not be used.
- In the winter months, when the flu infection is high and the risk of transmission is high, a limited time should be spent in closed and crowded environments to protect from influenza infections.
- To avoid infection. Seasonal flu vaccine and pneumonia vaccine should be given in consultation with the doctor.
- A proper diet should be made as the use of corticosteroids may cause blood sugar irregularities.
- A balanced diet should be taken care of by consuming enough from each food group.
- The use of tobacco products and excessive alcohol consumption should be avoided.
- Care should be given to body and oral hygiene and necessary importance should be given.
- If there are no objectionable conditions, regular daily exercise should be done.
- In mild cases, topical cortisone creams may be sufficient. In order to prevent infection of the superficial wounds caused by the opening of the blisters, it is appropriate to keep them clean and to use wound-healing topical treatments.