Majority of the individuals affected with pemphigus vulgaris often develop the first blisters in the mouth. This then spreads to the other neighboring regions such as nose, throat, and conjunctivae [6], and genitals (labia, vagina and cervix) [7].

• Difficulty in eating and swallowing food due to blisters in the throat and mouth
• Pain in the affected area 
• Pus can ooze from the blisters
• Dystrophic nails due to chronic paronychia and subungal hematoma caused by pemphigus vulgaris [8]

• Malnutrition

  A positive Nikolsky sign (induction of blistering in normal skin or at the edge of a blister) is indicative of the disease. [5] Severe pain with chewing can lead to weight loss and malnutrition. [5] Pathophysiology [ edit ] Pemphigus is an autoimmune [en.wikipedia.org]

  Complications Possible complications of pemphigus include: Infection of your skin Infection that spreads to your bloodstream (sepsis) Malnutrition, because painful mouth sores make it difficult to eat Medication side effects, such as high blood pressure [mayoclinic.org]

  INTRODUCTION Pemphigus vulgaris is a potentially fatal autoimmune disease in which severe blistering of the skin and mucous membranes appears that can lead to malnutrition and sepsis.[1] Systemic immunosuppression with a combination of systemic glucocorticoids [jsstd.org]

  One patient refused to pursue the medication and died of malnutrition, 6 months after diagnosis. One patient developed an extended form of pemphigus and died of MRSA sepsis, 7 months after diagnosis. [ojrd.biomedcentral.com]

  Left untreated, pemphigus vulgaris was uniformly fatal within 5 years due to severe blistering of the skin and mucous membranes leading to malnutrition, dehydration, and sepsis. Treatment options are summarized in Table I. Table I. [dermatologyadvisor.com]

• Cushingoid

  Her depressed mood, osteoporosis, diabetes mellitus, cushingoid habitus, and increased likelihood of infections in view of recent surgery precluded use of corticosteroids and motivated us to vigorously counsel the reluctant caretakers regarding the inescapable [mjdrdypu.org]

  Adverse Effects Adverse effects from corticosteroids reported in these papers included Cushingoid symptoms, diabetes mellitus, osteoporosis, hypertension, insomnia, GI upset, increased weight, candidiasis, tuberculosis, mood change, abnormal liver function [doi.org]

  Of concern was the high rate of serious side‐effects, with cushingoid features in 65%, growth retardation in 50% and infection in 50%.267 Juvenile PV has features similar to adult PV, but disruption of biological and social development due to the skin [onlinelibrary.wiley.com]

• Pediatric Disease

  Rituximab, an anti-CD20 monoclonal antibody, is an important medication in adult PV but has rarely been used to treat pediatric disease. [ncbi.nlm.nih.gov]

• Melena

  We present a unique case of EDS presenting with melena in which diagnostic endoscopy enabled to diagnose and establish link with patient's remote history of PV. [ncbi.nlm.nih.gov]

• Conjunctival Hyperemia

  The most common clinical signs were conjunctival hyperemia (87.5 %), erosions on the eyelids (41.6 %) as well as of the palpebral/bulbar conjunctiva (33.3 %) and at the medial epicanthus (20.8 %). [ncbi.nlm.nih.gov]

• Medial Epicanthus

  The most common clinical signs were conjunctival hyperemia (87.5 %), erosions on the eyelids (41.6 %) as well as of the palpebral/bulbar conjunctiva (33.3 %) and at the medial epicanthus (20.8 %). [ncbi.nlm.nih.gov]

• Photophobia

  The most relevant associated symptoms included local pain/stinging (71.4 %), irritation (47.6 %), photophobia (38.1 %), and epiphora (23.9 %). Ocular PV improved with systemic and adjuvant topical therapies. Only two patients experienced sequelae. [ncbi.nlm.nih.gov]

• Blister

  The resultant reaction is the formation of blisters. [symptoma.com]

  About 50% of people with this condition first develop painful blisters and sores in the mouth. This is followed by skin blisters. Skin sores may come and go. [nlm.nih.gov]

  Desmosomes at blister sites were occasionally split, with PV IgG decorating the extracellular faces of split desmosomes. [ncbi.nlm.nih.gov]

  Specialty Dermatology Pemphigus vulgaris is a rare chronic blistering skin disease and the most common form of pemphigus. Pemphigus was derived from the Greek word pemphix, meaning blister. [en.wikipedia.org]

• Nikolsky's Sign

  The blisters were suspected to represent the Nikolsky's sign. The histological findings of her skin were characterized by suprabasal acantholysis and mixed inflammatory cell infiltrates, including scattered eosinophils. [ncbi.nlm.nih.gov]

  Different types of Nikolsky’s sign: Marginal Nikolsky’s sign: Erosion of perilesional skin on the application of pressure is called marginal Nikolsy s sign. This sign is 69 % sensitive in detecting PV. [lecturio.com]

  […] of epidermal cells intraepidermal blister direct immunofluorescence intercellular deposition of IgG or C3 in net-like (reticular) pattern Differential Bullous pemphigoid less severe than PV does not affect mucous membranes negative Nikolsky sign Treatment [step2.medbullets.com]

  Extraoral examination of the skin of hand revealed multiple bullae (Figure 1B, black arrows) with positive Nikolsky sign 1 and Asboe—Hansen sign or Nikolsky sign 2. [academic.oup.com]

  Theoretically, the blisters should demonstrate a positive Nikolsky's sign, in which the skin sloughs off from slight rubbing, but this is not always reliable. [en.wikipedia.org]

• Eczema

  Types & treatments Childhood eczema Adult eczema Insider secrets Types of hair loss Treatment for hair loss Causes of hair loss Hair care matters Insider secrets What is psoriasis Diagnosis & treatment Skin, hair & nail care Triggers Insider secrets What [aad.org]

  This may easily be mistaken for more common problems such as eczema or fungal infections or seborrhoeic dermatitis, but does not respond to treatments for these conditions. It may spread to the body and become painful. [dermcoll.edu.au]

  The lack of blisters and mucosal involvement means diagnostic delay is frequent, as PF can be misdiagnosed as eczema, seborrhoeic dermatitis, actinic keratoses or psoriasis. [onlinelibrary.wiley.com]

  Sometimes pemphigoid may look like hives or eczema without blisters. [rarediseases.org]

  Impetigo, impetigenous eczema- most patients of pemphigus foliaceus in literature were initially diagnosed as impetigo 3. Erythroderma- patients of pemphigus foliaceus initially presenting as erythroderma have been reported. [e-ijd.org]

• Thin Skin

  Side effects If taken for a long time at high doses, steroid medication can have a range of unpleasant side effects, such as: increased appetite and weight gain thin skin that can bruise easily acne increased risk of infections mood changes and mood swings [nhs.uk]

  Long-term corticosteroid use If you are required to take steroid medication (corticosteroids) on a long-term basis (more than three months) the side effects include: further weight gain thinning skin that can bruise easily muscle weakness a combination [your.md]

• Neglect

  Currently the common use of pulse corticosteroids, often in first-line treatment, is being neglected. [ncbi.nlm.nih.gov]

  Furthermore, extrapolation from the reported literature continues to emphasize the neglected painful nature of pemphigus equally in periungal and mucocutaneous presentations [31]. [escholarship.org]

It can sometimes get difficult to diagnose pemphigus vulgaris as blisters can accompany some other disease condition as well. Physical examination to carefully study the blisters is necessary. In addition, the following procedures are carried out to diagnose the condition:

• Nikolsky sign for skin peeling: In this test, the area around the affected region is rubbed off with finger or cotton swabs. If the skin peels then it is a sign of pemphigus vulgaris. 
• Skin biopsy: It is an additional test required for confirming the diagnosis. In this, a sample of skin from the affected area is tested under the microscope. Plucked hairs direct immunofluorescence may substitute for skin biopsy and may grant similar yield [9].
• Blood tests: Blood tests are done to analyze the levels of desmoglein antibodies. Elevated levels of this antibody are the sign of pemphigus vulgaris.
• Direct immunofluorescence from perilesional skin areas demonstrates the in vivo deposition of antibodies [10].

• Listeria Monocytogenes

  Immunocompromised patients are at increased risk for developing meningitis or, rarely, brain abscess with opportunistic organisms like Listeria monocytogenes. [ncbi.nlm.nih.gov]

In severe cases, hospitalization may be required. Wound management may be required and the patient would be kept in separate intensive care units. The primary goal of the treatment is to relieve the symptoms and prevent the spread of infections to other parts of the body. The following are the methods employed to treat pemphigus vulgaris.

• Intravenous administration of fluids and electrolytes
• If mouth ulcers are severe then IV feeding may be required
• Administration of strong antibiotic regime to prevent spread of the infection
• Immunosuppressants to control the abnormal functioning of the immune system
• Pain relieving medications to reduce pain due to the blisters
• Anesthetic mouth lozenges may also be given for relieving the pain due to mouth ulcers
• Therapeutic plasmapheresis is a method that separates the plasma component from the blood with the aim of removing all the antibodies. The lost plasma is then replenished through intravenous fluids.

If the disorder is diagnosed on time and treatment initiated then it can be controlled. Failure to do so shall cause life threatening consequences with development of severe infection which finally leads to death. Pemphigus vulgaris carries a 5 – 15% mortality rate in general [5].

Complications

Complications can occur if the disorder is not well managed with appropriate treatment regime. The following is a list of complications of pemphigus vulgaris:

• Severe secondary skin infections
• Sepsis characterized by spread of the infection
• Dehydration that tends to get severe
• Debilitating side effects of the medications such as immunosuppressants and corticosteroids prescribed for controlling the blisters
• Death due to uncontrolled severe infection

Pemphigus vulgaris is an autoimmune disease, wherein the immune system of the body regards the proteins that binds the cells of the skin together as invaders and attacks them by producing antibodies. The resultant reaction is the formation of blisters. Other causes, though rare, but known to give rise to pemphigus vulgaris include the following:

• Medications belonging to the class of ACE inhibitors prescribed for patients with high blood pressure are known to trigger the development of pemphigus vulgaris.
• Another drug known as penicillamine is also known to play foul in the causation of such a type of skin disorder.

Pemphigus vulgaris is a rare condition, affecting about 1 to 5% of people in the UK. There is only a 1.3 incidence per million population in France with this cutaneous disease [1]. It has also been estimated that every 3 in 100, 000 individuals are affected by this skin disorder. It is not an infectious disease and does not spread by contact.

People belonging to Mediterranean region and Indian subcontinent are more prone to develop this skin disorder. The Jewish population is more prone to Pemphigus vulgaris than any other races in the world [2]. The mean age of onset is 50-60 years old in western nations but it is considerably younger in India [3].

Under normal circumstances, the immune system of the body attacks the foreign bodies that invade the system. However, in case of autoimmune disorder, the body’s immune system regards the body cells as invaders and attacks them. Patients with active disease demonstrate autoantibodies of immunoglobulin G1 and immunoglobulin G4 subclasses in affected tissues [4]. Similarly, in pemphigus vulgaris, the immune system attacks the proteins of the skin and mucous membranes and facilitates the development of blisters. These blisters are tender to touch and very painful. The sites of occurrence of the blisters include the nose, mouth, throat and genital regions.

Pemphigus vulgaris cannot be prevented since it is an autoimmune disorder. However, the development of complications can certainly be kept at bay with early initiation of treatment.

Pemphigus vulgaris is an autoimmune disease of the skin characterized by development of painful blisters. In this, the immune system of the body regards the protein of the skin and the mucous membranes as invaders and attacks them causing development of painful skin lesions.

Pemphigus vulgaris can strike any individual at any age. However, middle aged individuals are frequently prone to contract such a type of skin disorder. Pemphigus vulgaris is a chronic skin disorder which can be controlled if treated on time.

Definition

Pemphigus vulgaris is a rare chronic skin disorder characterized by the development of blisters in the mouth, nose and throat region. In severe cases, the infection may spread to rest of the body and blisters can develop in the genital regions as well.

Cause

Pemphigus vulgaris is an autoimmune disease, wherein the body’s own immune system regards the proteins of the skin and mucous membrane as invaders and attacks them. The resultant reaction of such an activity is the development of sores and blisters on the skin.

Symptoms

The blisters primarily develop inside the mouth causing difficulty in eating. When it spreads to the throat region it causes difficulty in swallowing the food. The blisters are painful but do not itch. Pus may ooze out causing great discomfort for the individuals.

Diagnosis

A careful examination of the blisters is necessary followed by blood tests to check for the levels of antibodies. Skin biopsy test is done to analyze the affected skin under the microscope. A test known as Nikolsky’s skin is also carried out. In this test, skin around the affected area is rubbed with finger or cotton swabs. If the skin peels off it is a sign of pemphigus vulgaris.

Treatment

Treatment revolves around providing relief from the symptoms and controlling the infections as well. Antibiotics are administered through the intravenous route to control spread of infections. In case of severe mouth ulcers, the patient is given IVF feeds.

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10. Helander SD, Rogers RS 3rd. The sensitivity and specificity of direct immunofluorescence testing in disorders of mucous membranes. J Am Acad Dermatol. Jan 1994; 30(1):65-75.