Pemphigus Vulgaris (PV) is a chronic skin disorder that causes much discomfort to sufferers. Read and know what is Pemphigus Vulgaris as well as all about its causes, symptoms, diagnosis and treatment.
Pemphigus Vulgaris Definition
It is a chronic blistering skin disease that leads to the development of erosions (sores) and blisters on the skin surface and the mucus membranes. It is a rare autoimmune condition that is potentially severe.
Picture 1 – Pemphigus Vulgaris
The name “Pemphigus” comes from the Greek term “Pemphix” meaning “blister” or “bubble”.
Pemphigus Vulgaris Symptoms
The condition is mainly characterized by the development of chronic (persistent) skin lesions that are often painful in nature. In later stages, these may drain and ooze pus. Crusts may also develop over their upper surface, which can easily peel or come off.
Approximately half of all patients with this disorder first develop painful sores and lesions in the mouth. This is followed by the formation of blisters over the skin surface, which may come and go with time. In rare cases, sufferers may experience itchiness in these lesions.
The skin sores generally arises over a course of several days. The open lesions are typically located in the mouth or on other regions of the body, such as the scalp, neck and trunk (torso). Acute cases often lead to widespread developing of skin rashes that cover regions like the scalp, face (near the eyes) and the extremities. Lesions on the mouth can be extremely painful and discomforting, even without itchy symptoms. In later stages, these may get swollen and filled with pus. Eventually, the blisters rupture and release pus from within.
What Causes Pemphigus Vulgaris?
It is not exactly clear why these lesions develop in the first place. Some possible causes of Pemphigus Vulgaris are:
Genetic factors
Some medical researchers, however, have associated it with genetic factors and believe it to be a condition acquired through gene.
Age
The disease is also supposed to be related with increasing age. Though the condition may affect people of any age, it is found to be most common in middle-aged individuals or those who are over 60 years old. It is only in rare cases that the condition has been reported in children or adolescents.
Medications
In rare cases, PV can arise due to use of immunosuppressive drugs or blood pressure medications. Blood pressure medicines, known as ACE inhibitors, are commonly suspected to result in this condition. A drug known as Penicillamine, which acts as a chelating agent and moves out certain substances from the bloodstream, is also believed to lead to this problem.
Autoimmune disorders
PV is an autoimmune disease. People suffering from already existing autoimmune conditions are more likely to have this disorder.
In healthy individuals, Desmoglein proteins bind the cells in the mucous membranes and skin together. In people with PV, the immune system produces antibodies specifically to fight desmoglein proteins. The antibodies are released into the bloodstream, which travel to the cells and attack desmoglein. The antibodies break the bonds existing between skin cells and lead to the weakening of the outer tissue layers. The affected skin region becomes extremely delicate and even gently rubbing it can make it red and cause it to break. This gives rise to blisters on the skin surface.
Pemphigus Vulgaris Diagnosis
Dermatologists begin the diagnosis of this condition by simple observation of the physical appearance of blisters. The skin is also rubbed to check if it breaks apart. If PV is present, rubbing the unaffected skin surface sideways with a finger or a cotton swab can make the skin separate easily. This technique is known as a positive Nikolsky’s sign.
Generally, a skin biopsy is performed to confirm the diagnosis. Samples of skin tissues and blood are usually collected and examined in a laboratory to detect presence of specific antibodies. Biopsy of the mucous membrane and direct immunofluorescence can also help in confirming the diagnosis.
Pemphigus Vulgaris Differential Diagnosis
The differential diagnosis of Pemphigus Vulgaris involves distinguishing it from other variants of Pemphigus, such as:
- Pemphigus foliaceus
- Pemphigus erythematosus
- Pemphigus vegetans
- Paraneoplastic pemphigus
The disease should also be differentiated from other clinically similar conditions, such as:
- Herpes simplex lesions
- Bullous pemphigoid blisters
- Dermatitis herpetiformis lesions
- Erythema multiforme blisters
- Lichen planus lesions
Pemphigus Vulgaris Treatment
It is extremely essential to treat this disease. This is due to the fact that the condition is likely to get progressively worse and more painful in the absence of medical attention. Typically, patients with mild symptoms are treated with protective bandages and topical corticosteroids in the office of a dermatologist. Antibiotics, antifungal medications and anti-inflammatory drugs may be prescribed to control, combat or even prevent infections.
Patients with severe and extremely painful skin problems are generally hospitalized. Intravenous fluids, electrolytes and corticosteroids are administered to alleviate the symptoms. Often, topical anesthetics (such as mouth lozenges) are provided to ease painful sensations on the skin and in the mouth. If there are acute mouth ulcers, patients should be fed in an intravenous (IV) manner.
If the condition fails to improve, blood or plasma transfusions should be considered as a last resort. The disease should be treated as an emergency condition and patients should be cared after in intensive care units until completely cured.
Treatment helps alleviate pain and other symptoms and prevent complications, particularly infections.
Pemphigus Vulgaris Medicines
If localized pain relief is not found to be enough in easing body aches, analgesics (pain-relieving medications) may be used. Systemic (body-wide) treatment is required to control the discomforting symptoms and should be started as early as possible. Systemic therapy includes use of:
- Corticosteroids
- Drugs containing gold
- Dapsone (an anti-inflammatory drug)
Oral corticosteroids are the primary curative agent for this disorder. Ever since they have been used, mortality rate associated with this condition has dropped to 5-15% from a staggering 99%. Immuno-suppressive medications (drugs that suppress the immune system) may also be used for cure. These include brands like:
- Azathioprine
- Methotrexate
- Cyclosporine
- Cyclophosphamide
- Mycophenolate mofetil
- Rituximab
While systemic therapy can be effective, it can produce serious side effects that may give rise to complications.
Certain antibiotics, especially doxycycline and minocycline, are also found to be effective in curing the problem. Occasionally, patients are treated with Intravenous Immunoglobulin (IVIg).
In some cases, systemic therapy may be accompanied by Plasmapheresis, a process that involves removal of plasma (containing antibodies) from the bloodstream and replacing it with donated plasma or intravenous fluids. Plasmapheresis helps in reducing the number of antibodies in blood.
Wet dressings, drying lotions and similar measures used for blister and ulcer treatments are also considered to provide PV sufferers with a soothing sensation.
Pemphigus Vulgaris Natural Treatment
For curing Pemphigus Vulgaris, natural remedies have also shown to yield effective results. These include:
Apples
Daily consumption of three apples or two glasses of apple juice can be effective in curing the symptoms of Pemphigus Vulgaris. Daily intake of applesauce can also yield effective results. People may also pour applesauce into a food dehydrator and make homemade fruit leather.
Ayurveda
Herbal applications of jatyadi oil, chandanadi oil, yashtimadhuk-ghrut, shatadhout-ghrut and panch-tikta-ghrut can also be used for treatment. An ointment comprising of chandan, haridra, mandukparni, manjishtha and saariva as ingredients can also be used.
Acupuncture
Patients suffering from nausea due to PV may be treated with Acupuncture. Acupuncture helps in curing pain, nausea as well as side effects of treatment in PV patients. People having inhibitions with penetration of the skin may try electro-acupressure instead.
Herbs
Mix calendula, tea tree oil, thyme and lavender. Clean the affected regions with mild soap. Then directly apply the mixture onto the skin and cover the area with a sterile bandage. However, it is best to consult your doctor before using any such remedies.
Pemphigus Vulgaris Prognosis
Without treatment, this condition is usually life-threatening. Severe infection is the most frequent cause of death. With treatment, the disorder tends to be chronic. Side effects of treatment may be severe or disabling.
Pemphigus Vulgaris Complications
Some of the major possible complications of this condition include:
- Severe dehydration
- Medicinal side effects
- Secondary skin infections
- Blood infection (Sepsis)
Sepsis involves spread of infection through blood and affects the entire body. It can give rise to life-threatening complications including respiratory difficulties in sufferers.
Pemphigus Vulgaris Life Expectancy
The mortality rate of this condition is within 5-15%. If treated properly in time, patients are not likely to suffer from any complications. Lifespan can be normal in such cases.
Pemphigus Vulgaris Prevention
It is not known whether the condition can be prevented from arising. However, worsening of the disease may be avoided by measures like:
- Minimizing activities that may traumatize the mucous membranes and skin during active phases of the disorder
- Consulting doctors before suddenly stopping use of medications, such as Corticosteroids
- Avoid eating hard or excessively spicy foods (in case of oral ulcers)
Pemphigus Vulgaris in Dogs
Pemphigus vulgaris, in case of dogs, is characterized by the development of severe ulcers on the skin around the nose, mouth, prepuce, vagina and anus. The mouth is affected in most cases. Secondary complications are likely.
Most dogs with PV lesions require lifelong use of immunosuppressant medications like Azathioprine and Tacrolimus along with oral corticosteroids, such as Prednisolone and Prednisone. Topical corticosteroids are also used for cure. Dietary changes, involving addition of more raw foods into the diet, have been found to be effective in improving the symptoms. Sunburn has been found to aggravate PV symptoms. Therefore, dogs with PV should be kept away from sunlight. Use of canine sun blocks is also recommended.
Is Pemphigus Vulgaris Contagious?
No, this is not a contagious condition. Humans or animals affected with PV cannot transmit it to other unaffected creatures. It is not caused by a bacterial or viral infection. A person cannot contract PV by touching the blisters formed by this syndrome.
Is Pemphigus Vulgaris Genetic?
PV sufferers often worry whether their children may also suffer from the condition. Actually, this can happen very rarely. Even though genetic factors are possibly involved in the development of PV, it is not hereditary. It is only in the rarest of rare cases that more than one member of the same family has been diagnosed with PV.
Pemphigus Vulgaris Vs Bullous Pemphigoid
Pemphigus Vulgaris is often mistaken for Bullous Pemphigoid (BP). These are two of the most common disorders in the group of autoimmune blistering conditions. Both are marked by the manufacture of autoantibodies that aim at the structural proteins essential for adhesion between cells and between cell and basement membrane. However, both have certain distinct characteristics. Pemphigus Vulgaris gives rise to large, flaccid bullae that rupture easily. Patients often develop ulcers and oral bullae in the early stages followed by the formation of bullae on the skin.
In Bullous Pemphigoid, the bullae are subepidermal in reality. Due to this reason, they are less fragile than the bullae formed in case of Pemphigus Vulgaris. The clinical appearance of the lesions varies a lot in this case. Unlike in PV, the ulcers in BP do not usually start in the mouth.
As aforementioned, PV can lead to life-threatening complications. Bullous Pemphigoid is, however, a less severe disorder. Often, there is no rupture of bullae involved. Naturally, there is less chance of scarring and infection.
Pemphigus Vulgaris Pictures
Looking for some good Pemphigus Vulgaris photos? Check out these useful Pemphigus Vulgaris images to know about the physical appearance of lesions caused by this condition.
Picture 2 – Pemphigus Vulgaris Image
Picture 3 – Pemphigus Vulgaris Photo
If you suspect yourself or any of your family members to be having this condition, call your health care provider immediately. You should also seek medical attention if you have been treated for PV symptoms and you are suffering from fever, chills, pain in joints or muscles, ulcers or a general feeling of illness. As aforesaid, early treatment is essential to make a faster recovery from this disease and evade the life-threatening symptoms that it may possibly produce.