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The pain of blisters in the mouth and skin: What are the initial signs of Pemfigos' disease?
Pemphigus vulgaris is a rare chronic blistering skin disease and is the most common form in the Pemfigus family.The name comes from the Greek word "pemphix", which means blisters.The disease is classified as a type 2 hypersensitivity reaction, characterized by the formation of antibodies on the adhesion structure of the skin: desmosomes, which are responsible for keeping certain layers of the skin bonded to each other.When these deadherent bodies are attacked, the skin layer begins to separate, and the clinical characteristics are similar to the formation of vesicles.These vesicles are caused by the disintegration of intercellular junctions (called "acantholysis", a process that is caused by autoantibodies-mediated reactions.Furthermore, the disease will inevitably progress without treatment, and the size and distribution of the lesions will increase over time, showing physiological characteristics like severe burns.
Currently, through modern treatments, the mortality rate of Pemfigos disease has dropped from nearly 90% to 5% to 15%.
The main victims of Pemfigos disease are mostly between the ages of 50 and 60 years, and the incidence is relatively high in women.This disease often first appears in the oral cavity, which is commonly found in the mucosa of the cheeks and soft jaws.However, in addition to the mouth, other mucosal surfaces such as the conjunctiva, nose, esophagus, penis, vagina, cervix and anus may also be affected.Loose blisters often appear on the skin, while palms and soles of the feet are usually unaffected.Eventually, these blisters can rupture and cause ulcers and erosion.
According to research, Pemfigos disease is an autoimmune disease caused by antibodies made against deadherents (desmoglein 1 and desmoglein 3).The presence of these antibodies reduces the cohesion of keratinocytes in the epidermis and destroys the protective function undertaken by the intact skin.
In histological examination, the keratinocytes in the basal layer are usually still attached to the basal membrane, and the characteristic phenomenon is called "tombstoning".
The diagnosis of Pemfigos disease is often challenging.One of the early signs is ulcers in the mouth or blisters in the skin, which can be accompanied by itching or pain.In theory, these blisters should exhibit positive Nikolsky's sign, where the skin falls off under a slight friction, but this is not always reliable.The classical diagnostic method is to perform a pedicle examination around the lesion and use direct immunofluorescence staining technology to test, in which skin cells lacking normal cell-to-cell junctions can be observed, namely the so-called spinous fusion cells.
In terms of treatment, corticosteroids and other immunosuppressants have been used to alleviate symptoms of Pemfigos disease.However, the use of corticosteroids may be accompanied by severe and lasting side effects and should be restricted as much as possible.Monoclonal antibodies such as rituximab are considered effective alternatives to corticosteroids and are increasingly used as first-line treatments.With the FDA's approval of rituximab in 2018, results from many cases suggest that patients can achieve remission after a course of treatment.
Study shows that if treatment is initiated early, the efficacy of Pemfigos disease will be more obvious.
Pemfigos disease is a relatively rare disease with only about 1 to 5 cases per million people.In the United States and other major markets, the efficacy of treatment varies from patient to patient. For example, for some patients, there may be only mild lesions, but others may experience more severe symptoms.Therefore, doctors are still working to find effective treatments to reduce the side effects of corticosteroids during treatment.When faced with this autoimmune disease, timely professional diagnosis and treatment will be the key to improving patients' quality of life.
So, what further measures can the medical community take to improve the patient's condition for this disease that may cause pain and low quality of life?
