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]

• Inflammation

  NSAIDs not only help to manage pain, but they block a specific protein that causes inflammation. The second option is something called corticosteroids. Corticosteroids are a natural hormone that your body produces in response to inflammation. [fibromyalgiatreating.com]

  However, severe inflammation may induce reactive epithelial cell changes and hence mimic histologic dysplasia. [ncbi.nlm.nih.gov]

  However, should inflammation occur, it can fill with fluid. Inflammation of the lining of the heart is called pericarditis (itis=inflammation). [medicinenet.com]

• Sepsis

  We describe a case of a patient with pemphigus who developed a sepsis due to Citrobacter freundii infection. [ncbi.nlm.nih.gov]

  Because the disease remained active and she was in sepsis with persistent fever, we could not consider giving her intravenous corticosteroids. [jmedicalcasereports.biomedcentral.com]

  The skin lesions may at times become infected and may lead to systemic sepsis. The treatment for PV should include hospitalization of the patient who is ill, septic, or unable to take oral fluids. [journals.lww.com]

  Sepsis was associated with 100% of the deaths. CONCLUSIONS: The occurrence of the disease is rare, there are no familiar/endemic outbreaks in the sample. Evolution is usually favorable, but secondary infection is associated with worse prognosis. [scielo.br]

• Weight Loss

  This case report presents a 13-year-old boy referred to the Department of Paediatric Dentistry, Women's and Children's Hospital, Adelaide, South Australia, Australia, with a 5-week history of severe oral ulcerations and significant weight loss of unknown [ncbi.nlm.nih.gov]

  Those in the mouth can interfere with eating and drinking and can lead to weight loss, hence the advice of the dentist would help especially those with dentures. [pemfriends.co.uk]

  She reported unquantified weight loss and an oral mucosa swab was performed during the investigation for fungus research, with a positive result. [karger.com]

  Pemphigus vulgaris and its treatments can be debilitating and may cause lost time at work, weight loss, loss of sleep, and emotional distress. [rarediseases.info.nih.gov]

• Malnutrition

  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]

  […] sports) if you have blisters on your skin Complications of pemphigus vulgaris Pemphigus vulgaris can lead to other problems, such as: blisters and sore patches becoming infected, which can cause life-threatening problems such as sepsis weight loss and malnutrition [nhs.uk]

  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 disease [en.wikipedia.org]

  Chewing and swallowing can worsen pain and lead to malnutrition and weight loss. The ruptured skin blisters may also limit a person’s daily activities. [medicalnewstoday.com]

• Pallor

  […] accompanied by abdominal pain, aphthae in oral mucosa, asthenia, weight loss, and anorexia Folic acid or vitamin B12 deficiency Oral pain, erythematous tongue, asthenia and anemia, paresthesias in limbs, and physical problems Hypochromic iron deficiency Pallor [omicsonline.org]

  […] accompanied by abdominal pain, aphthae in oral mucosa, asthenia, weight loss, and anorexia Folic acid or Vitamin B12 deficiency – Oral pain, erythematous tongue, asthenia and anemia, paresthesias in limbs, and physical problems hypochromic iron deficiency pallor [jdrr.org]

• Pneumonia

  Pneumocystis carinii pneumonia and septicemia were found as the two fatal and serious adverse events associated with rituximab. Moreover, development or reactivation of herpes simplex and herpes zoster and cytomegalovirus should be warned. [ncbi.nlm.nih.gov]

  and Staphylococcus aureus [ 16 ], one bacterial pneumonia [ 22 ], one recurrence of P. aeruginosa hip arthritis [ 22 ], one severe late-onset neutropenia after 27 weeks [ 28 ], one late-onset neutropenia and bacterial pneumonia after 19 weeks [ 29 ] [link.springer.com]

  Serious infections occurred in 6 patients (9%) in the rituximab group (pneumonia and upper respiratory tract infection, cellulitis and acute pyelonephritis, pyelonephritis, viral pneumonia, infective bursitis, and skin infection) and in 4 patients (6% [nejm.org]

  Pericarditis is often the result of an infection such as: Viral infections that cause a chest cold or pneumonia Infections with bacteria (less common) Some fungal infections (rare) The condition may be seen with diseases such as: Cancer (including leukemia [medlineplus.gov]

  Some of the documented adverse effects include severe infections such as pneumonia, progressive multifocal leukoencephalopathy, anaphylaxis, Stevens–Johnson syndrome. [journals.lww.com]

• Diarrhea

  She reported blisters in the mouth at the onset, apart from thrush, drooling, dysphagia, intermittent fever, and diarrhea. She felt her problems were related to her orthomolecular diet, and gave a history of gastric ulcers and genital herpes. [arquivosdeorl.org.br]

  Side effects include abdominal pain, vomiting, and diarrhea. Corticosteroids: If neither of the first two options have worked, corticosteroids may be employed. [medicalnewstoday.com]

  Hypochromic iron deficiency Pallor, fatigue, cephalalgias, vertigo, buzzing in the ears, irritability, insomnia, concentration problems, sensitivity to cold, anorexia and nausea Enteropathic acrodermatitis Loss of taste and smell, sight problems, intense diarrhea [omicsonline.org]

  […] hypochromic iron deficiency pallor, fatigue, cephalalgias, vertigo, buzzing in the ears, irritability, insomnia, concentration problems, sensitivity to cold, anorexia, and nausea Enteropathic acrodermatitis – loss of taste and smell, sight problems, intense diarrhea [jdrr.org]

  Common side effects include nausea, vomiting, diarrhea, and abdominal discomfort. [statpearls.com]

• Oral Ulcers

  This case report presents a 13-year-old boy referred to the Department of Paediatric Dentistry, Women's and Children's Hospital, Adelaide, South Australia, Australia, with a 5-week history of severe oral ulcerations and significant weight loss of unknown [ncbi.nlm.nih.gov]

  MeSH Key Words: case study; oral ulcer/etiology; pemphigus/drug therapy; pemphigus/immunology Reply to this article | View replies [0] [cda-adc.ca]

  Keywords Pemphigus vulgaris; Oral ulceration; Demoglein; Autoimmune Introduction Autoimmune blistering conditions are an uncommon cause of oral mucosal ulceration. [esciencecentral.org]

  She complained of persistent painful oral ulcers that had appeared approximately nine months previously. Small vesicles had been noted by the patient prior to the development of the oral ulcers. [scielo.org.za]

• Aphthous Stomatitis

  The most frequent diagnoses in patients with oral lesions are recurrent aphthous stomatitis, Behçet disease, erythema multiforme, erosive lichen planus, and oral candidiasis [ 2 ]. [omicsonline.org]

  The differential diagnosis of acute herpetic stomatitis and recurrent aphthous stomatitis was suggested. Biopsy was done, and the tissue was sent for histopathological examination. The oral tissue biopsies were taken from the perilesional site. [jdrr.org]

  Keywords Immunosuppressive Drug Bullous Pemphigoid Oral Cyclophosphamide Recurrent Aphthous Stomatitis Clobetasol Propionate These keywords were added by machine and not by the authors. [link.springer.com]

• Aphthous Ulceration

  Differential diagnosis The differential diagnoses will include other blistering eruptions of the skin and mucous membranes: Aphthous ulcers (oral lesions). Herpetic lesions. Bullous pemphigoid. Impetigo. [patient.info]

  Referred to as a differential diagnosis, the investigation may include such diseases as: Aphthous ulcers Erysipelas Erythema multiforme Lupus Oral lichen planus Pustular psoriasis Stevens-Johnson syndrome (SJS) Toxic epidermal necrolysis (TEN) Treatment [verywellhealth.com]

  Differential Diagnosis of Pemphigus Vulgaris Differentials for mucosal lesions: Acute herpetic stomatitis, erythema multiforme, aphthous ulcers, lichen planus or mucous membrane pemphigoid. [lecturio.com]

• Sialorrhea

  Other oral manifestations include sialorrhea, halitosis, and the continuous formation of brown or blackish crusts at the vermillion border [ 1, 18 ]. Figure 4: Involvement of free and attached gingiva in PV. [omicsonline.org]

• Blister

  Pemphigus vulgaris is an uncommon, potentially fatal, autoimmune disorder characterized by intraepidermal blisters and extensive erosions on apparently healthy skin and mucous membranes. [msdmanuals.com]

  Early in the disease patients may have erosions in the mouth or blisters on the skin. These blisters can be itchy or painful. [en.wikipedia.org]

  Autoantibodies against desmoglein (specifically, Dsg 3) cause the intraepidermal blistering seen in pemphigus, while autoantibodies against hemidesmosomes are the reason for the subepidermal blistering seen in pemphigoid. [clinicaladvisor.com]

  Most patients get erosions in their mouth at some time; but some never get blisters or erosions on their skin. [pemfriends.co.uk]

  This picture shows a close-up of the blistering on the back. Most of the blisters have broken (denuded), which is common since these blisters are fragile. [mountsinai.org]

• Nikolsky's Sign

  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]

  Nikolsky’s sign Nikolsky`s sign was first described by a Russian dermatologist Pyotr Vasilievich Nikolskiy. [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]

  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]

  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]

• Eruptions

  One year after onset of mucosal lesions, she developed an abdominal eruption with several tense vesicles and bullae on an erythematous base. The hematoxylin and eosin stain sample was consistent with a diagnosis of pemphigus vulgaris. [ncbi.nlm.nih.gov]

  References Case Report A 60-year-old man presented with eroded areas in the mouth and blistering eruptions on the scalp, face, trunk, arms, and legs. [mdedge.com]

  Our patient showed the classical symptoms of pemphigus vulgaris, such as a generalized bullous skin eruption first starting under the breasts and in the axillae, then developing on the abdomen and later extending to the extremities. [jamanetwork.com]

• Oral Blisters

  Here, the authors report a case of a woman who sought for treatment of generalized oral blisters and ulcers that caused severe pain. She had undergone a previous biopsy by other professionals that was inconclusive. [ncbi.nlm.nih.gov]

  Oral blisters have a very thin roof and readily rupture due to oral traumas, giving rise to multiple chronic painful bleeding ulcers and erosions that heal with difficulty [ 5, 7, 8, 13 ]. [omicsonline.org]

  A small number of patients will present with cutaneous blistering first; however, all will go on to develop oral lesions. Pemphigus foliaceus (PF): Presents with lesions on the skin only, and these patients will not go on to develop oral blisters. [patient.info]

  There has historically been a higher incidence in women.[4] Signs and symptoms[edit] Pemphigus vulgaris most commonly presents with oral blisters (buccal and palatine mucosa, especially), but also includes cutaneous blisters. [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]

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

  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]

  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]

• Asthenia

  Oral pain, erythematous tongue, asthenia and anemia, paresthesias in limbs, and physical problems Hypochromic iron deficiency Pallor, fatigue, cephalalgias, vertigo, buzzing in the ears, irritability, insomnia, concentration problems, sensitivity to [omicsonline.org]

  – Oral pain, erythematous tongue, asthenia and anemia, paresthesias in limbs, and physical problems hypochromic iron deficiency pallor, fatigue, cephalalgias, vertigo, buzzing in the ears, irritability, insomnia, concentration problems, sensitivity to [jdrr.org]

  Other effects manifesting with greater intensity comprise hypertension, hyperglycemia, blurry vision with the development of glaucoma, and posterior subcapsular cataracts, palpitations, swelling of the legs, malaise and asthenia. [elsevier.es]

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].

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.

1. Bastuji-Garin S, Souissi R, Blum L, et al. Comparative epidemiology of pemphigus in Tunisia and France: unusual incidence of pemphigus foliaceus in young Tunisian women. J Invest Dermatol. Feb 1995; 104(2):302-5.
2. Pisanti S, Sharav Y, Kaufman E, Posner LN. Pemphigus vulgaris: incidence in Jews of different ethnic groups, according to age, sex, and initial lesion. Oral Surg Oral Med Oral Pathol. Sep 1974; 38(3):382-7.
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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.