Pemphigus vulgaris

An incapacitating condition, Pemphigus vulgaris (PV) usually displays localized symptoms for a few months before becoming fatal. Get complete information about this disorder, including its causes, symptoms, diagnosis and treatment.

Pemphigus vulgaris Definition

It is one of the rare types of chronic skin blistering disorder that can range from relatively mild to extremely painful and debilitating. The term “pemphigus” is derived from the Greek word pemphix meaning bubble or blister.

Pemphigus vulgaris Incidence

It is rarely observed in children and affects individuals above the age of 50. The disorder occurs in both males and females.

Pemphigus vulgaris Symptoms

Nearly 50-70% of all patients develop painful mucosal blisters as well as oral lesions at some point in the course of the disorder. These tiny, soft bubbles on the skin are filled with serum and represent the sole symptom of the condition. In certain patients, the oral lesions tend to become erosive, causing intolerable pain. The flaccid blisters make the skin appear wounded, causing extreme damage. Patients usually describe these skin sores as oozing, draining, peeling and crusting. The abscesses are commonly present in areas like:

Picture of Pemphigus vulgaris

Picture 1 – Pemphigus vulgaris

  • Face
  • Scalp
  • Groins
  • Armpits
  • Buttocks
  • Trunks

These blisters are rarely pruritic or itchy, but can erupt and turn into painful sores, leaving raw, red spots on the skin. The red sores then slowly undergo crusting and subsequently form scabs. The abrupt bursting can be highly attributed to the fragility of the delicate blisters. The affected areas of the skin do not go through scarring after healing. However, hyperpigmentation of the cutaneous layer could be seen in few patients. In some cases, individuals may develop only mouth blisters. Sufferers usually experience difficulties during activities like eating, drinking, speaking and brushing. The caustic sores can sometimes penetrate the inner areas of the skin, affecting mainly the anus, genitals, throat, vagina and retina. Blisters in the larynx or voice box can cause hoarseness. Swallowing turns painful when the condition occurs in the gullet.

Pemphigus vulgaris Causes

The dermatological disorder is an autoimmune blistering condition. It signifies an overactive immune response of the body against the cutaneous tissues. In other words, the body begins to attack the cells of the skin, causing blisters. The normal antibodies prepared by the immune system to destroy the harmful microorganisms suddenly begin to attack a particular part of the body in an autoimmune disorder. The tissues of the skin compose of an essential protein, which facilitates binding of the epidermal cells. In this autoimmune condition, specific antibodies are made against this protein that weakens the binding force between the skin cells. After complete breakage of the bond, the cells begin to detach from each other. On dissociation, fluid begins to occupy the spaces between the separated cells that slowly develop into afflictive blisters.

The predominant reason behind the occurrence of PV is still unknown. The disorder could be an inherited condition, making some individuals more vulnerable to blisters. Medical investigators have considered some possible triggers that could abnormally activate the immune system to destruct the skin tissues. Some of these may include:

  • Viruses
  • Infectious agents
  • Environmental elements

Pemphigus vulgaris Pathophysiology

Desmosomes form a type of cell-cell junction- a multiprotein complex that provides contact between neighboring cells. These intracellular bridges are present between epithelial, myocardial and other cell types. Both desmoglein 1 and desmoglein 3 form the calcium-binding transmembrane glycoprotein component of desmosomes in the epithelial cells. In this autoimmune disorder, the antibodies are produced against these proteins, causing loss of cohesive force between the keratinocytes- the epidermal basal cells that synthesize keratin, responsible for providing strength to the skin. The basal keratinocytes do not detach from the basement membrane, which is a thin layer of fibers, underlying the epithelium. Therefore, in appearance the skin appears very rough and supple. Blisters are a result of accumulation of a low protein extravasular fluid called transudate between the keratinocytes and basement membrane.

Pemphigus vulgaris in dogs

PV encompasses a group of autoimmune disorders, in which there is blistering of the superficial skin and/or mucosal layers, and Pemphigus vulgaris is one of its constituent, which is quite rare in dogs. However, these canine animals can develop another form of the autoimmune blistering disorder called Pemphigus foliaceus. It is generally characterized by formation of cluster of lesions on both skin and mucous membrane. The small vesicles eventually evolve into pustules that may rupture, forming erosions.

Pemphigus vulgaris Diagnosis

Skin care experts can conduct a better evaluation of the condition with the help of the following diagnostics tests:

Skin biopsy

The test requires removal of a piece of skin for the purpose of further examination using a microscope. It is necessarily performed to identify the exact cause of blisters.

Immunofluorescence

Indirect immunofluorescence is a highly specialized laboratory technique that measures the amount of antibodies produced against the epidermal protein in the blood. The level of the auto-antibodies also determines the severity of the disorder. On the other hand, direct immunofluorescence involves staining of the skin biopsy with a dye to detect the antibodies in the epidermal cells.

Pemphigus vulgaris Differential Diagnosis

The presence of fluid-filled blisters or bullae can often be confused with innumerable types of skin disorders. Physicians must conduct the required tests to eliminate the possibility of conditions like:

  • Bullous pemphigoid
  • Pemphigus foliaceus
  • Bullous contact dermatitis
  • Erythema multiforme
  • Dermatitis herpetiformis

Pemphigus vulgaris Treatment

The primary treatment of PV aims at alleviating the painful symptoms caused due to the blisters and ensures rapid healing of the localized lesions. Severe cases of the condition require proper management of the skin condition as the consequences could prove to be fatal. The therapeutic interventions commonly used for the blistering skin disorder involves the following:

Topical medication

A wide variety of ointments, sprays and mouthwashes, consisting of a moderate dose of steroid can be regularly used to treat mouth blisters and erosions. Few mouthwashes contain antiseptic or local anesthetic to compress the pain. The raw, bright areas on the skin can be treated with first aid measures such as dressings. Antifungal and antibiotic drops or pastilles promote elimination of infection in the mouth, throat or gullet.

Steroids

The medication can be taken in the form of tablets such as prednisolone. Steroids are usually prescribed to reduce the redness and soreness of the skin. The drug treatment can produce effective results and the affected patients begin to improve within few days. The dose usually varies from individual to individual. A corticosteroid injection can be administered to those patients who require higher doses of the medicine. Long-term use of steroids can give rise to a host of side effects like:

  • Muscle weakness
  • Weight gain
  • High blood pressure
  • Osteoporosis
  • Glaucoma
  • Delayed wound healing
  • Increased susceptibility to infections

Immunosuppressants

These medicines can be used in addition to steroid tablets for better results. The drugs dampen down the abnormal activity of the immune system and are quite powerful. The combination therapy can control the blisters to a greater degree. Some of the commonly used immunosuppressants include:

  • Methotrexate
  • Cyclophosphamide
  • Azathioprine
  • Cyclosporin
  • Mycophenolate mofetil
  • Gold
  • Tetracyclines
  • Nicotinamide
  • Dapsone

Plasmapheresis

It is a medical procedure performed outside the body and specifically carried out to eliminate the auto-antibodies in the blood, causing destruction of the skin cells. In this technique, the affected blood plasma is removed from circulation and replenished with intravenous fluid or plasma received from a donor. Although complete eradication of the self-destructive antibodies is not possible, it could reduce the negative effects of an overactive immune system.

Intravenous immunoglobulin

The technique is an attempt to decrease the severity of the autoimmune disorder in which a blood product, comprising of large amount of antibodies, is extracted from the plasma of several blood donors. The preparation is them administered intravenously to the affected patients.

Pemphigus vulgaris Complications

The erosions or blisters can gradually spread to the surrounding areas of the skin in the absence of appropriate treatment. The sore skin may get prone to a number of problems like:

Image of Pemphigus vulgaris

Picture 2 – Pemphigus vulgaris Image

  • Severe secondary infections
  • Metabolic disturbances
  • Dehydration

Pemphigus vulgaris Prognosis

In the past, mortality rate of the autoimmune disorder was very high, owing to the lack of awareness and unavailability of treatment options. With advancement in the medical field, the risk of death associated with the condition has now been reduced to a great extent. The blisters usually heal within a short span of time and the disorder remains in the inactive phase for a long period. However, severe side effects of the treatment can cause death in some patients.

Pemphigus vulgaris does carry a fatal chance and is more serious than the other types of blistering skin ailments. Treatment with steroid medicines and immunosuppressive drugs can help in the management of the condition. Affected patients should protect their skin and mouth from roughness. It is advisable to avoid contact sports and spicy foods to prevent skin flare ups. Proper use of ointments and consumption of bland food can reduce the soreness of the skin.

References:

http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001885/

http://en.wikipedia.org/wiki/Pemphigus_vulgaris

http://www.mayoclinic.com/health/pemphigus/DS00749

http://www.nhs.uk/Conditions/pemphigus-vulgaris/Pages/Definition.aspx

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