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Rare Skin Disease: Finding the Mysterious Roots of Pemphigus Disease!
Pemphigus vulgaris is a rare, chronic blistering skin disease and the most common form of Pemphigus disease. The term comes from the Greek word "pemphix," which means blister. Pemfeggs disease is classified as a type II hypersensitivity reaction, which occurs when antibodies attack the desmosome, a structural component of the skin, causing the skin layers to separate, forming Clinical manifestations similar to blisters. The blisters are caused by the shedding of epidermal cells due to an autoantibody-mediated reaction to intercellular junctions. If left untreated, the condition will continue to worsen, with blisters expanding throughout the body and pathological manifestations similar to those of severe burns. In the early days, the mortality rate of Pemphigos was as high as 90%. With the development of modern treatment, the mortality rate after treatment has dropped to between 5% and 15%, but in 1998, it was still the fourth leading cause of death from skin diseases, so the risk of death is still "potentially fatal". "of.
Symptoms and signs
The most common symptom of Pemfeggos disease is oral blisters, especially on the cheeks and palate; blisters on the skin may also occur. Other mucosal surfaces, such as the conjunctiva, nose, esophagus, genitals, and anus, may be affected. Patients often see tension-free blisters on the skin, while the palms and soles are usually spared. The blisters often rupture, leaving ulcers and erosive lesions. A positive Nikolsky sign is an important indicator of this condition, which means that light friction on normal skin or the edge of a blister will cause a blister to form. Sufferers may experience severe pain when eating, leading to weight loss and malnutrition.
PathophysiologyPemfegos is an autoimmune disease caused by antibodies against plaque adhesions. The loss of epidermal adhesion plaques results in loss of adhesion between epidermal cells and impairment of the skin's barrier function. This process is classified as a type II allergic reaction, in which antibodies bind to antigens from one's own tissues. On histological examination, keratinocytes of the basal layer often remain attached to the basement membrane, creating a characteristic known as a "tombstoning appearance." Exudate from the cavity accumulates between the stratum corneum and the basal layer, forming blisters that are easily detached when lateral force is applied, demonstrating a positive Nikolsky sign. The clinical features of this disease are extensive atonic vesicles and mucosal lesions, and the severity of the disease and the mucosal lesions are proportional to the level of exfoliated adhesion plaques.
Diagnosis
Because Pemfeggs disease is a rare disorder, diagnosis is often complex and time-consuming. In the early stages of the disease, people may develop blisters in their mouth or on their skin that may be itchy or painful. Nikolsky's sign of the blister should also be positive; however, this is not always reliable. The gold standard for diagnosis is a needle biopsy taken from the lesion area, which is examined after direct immunofluorescence staining and finds that the cells lack normal cell-cell connections. These cells are round-nucleated keratinocytes induced by antibody-mediated induction. Pemfege disease can be easily confused with impetigo and candidal infections. IgG4 is considered a pathogenic antibody.
TreatmentTreatment wise, steroids and other immunosuppressants are used to reduce the symptoms of Pemfeggs disease, but steroids can cause severe and long-lasting side effects, so their use should be limited. Intravenous immunoglobulin, acetaminophen, methotrexate, azathioprine, and cyclophosphamide have also been used with varying degrees of success. Monoclonal antibodies such as rituximab have gradually become the first choice of treatment and received full FDA approval in the summer of 2018. In many cases, many patients achieve remission after just one course of treatment. Starting treatment early in the disease process can yield better results. Long-term remission after treatment is also of interest.
EpidemiologyPemfeggs disease is a relatively rare condition, affecting approximately 1 to 5 people per million in the UK and 1 to 10 cases per million worldwide. In the United States, there are estimated to be more than 14,000 cases. In 2012, Israeli researchers said they had discovered a genetic cause for the disease, noting that Jews are 40 times more likely to develop the disease than other ethnic groups. The disease is not contagious and cannot be spread from person to person. Regardless of socioeconomic factors, the incidence of Pemfeggs disease does not differ significantly among different ethnic groups.
Research
Research on the use of genetically modified T cells to treat Pembrogliol disease was reported in 2016. Because rituximab indiscriminately attacks all B cells, this can weaken the body's ability to control infection. In the experiment, human T cells were genetically modified to specifically recognize B cells that produce anti-decapitation plaque antibodies. These studies open up new possibilities for future treatment directions.
The rarity of Pemfeggs disease means that research resources are limited, but the scientific community is still working hard to explore more effective treatments to improve the quality of life of patients. Faced with such a rare and complex disease, how can we find its deeper roots and treatment methods?
