Introduction
Skin and mucous membrane blistering are hallmarks of autoimmune bullous disorders of the upper aerodigestive tract. The term "pemphigus" refers to a category of autoimmune skin and mucous membrane blistering illnesses. Pemphigus vulgaris (PV), a bullous disease affecting the skin and mucous membranes, is a member of this category and may be fatal if not treated with the proper immunosuppressive medications.1
PV has an incidence of 0.1–0.5 cases per 100,000 people per year1 and equally affects both sexes. The average age of onset is between 50 and 60.2
Pemphigus vulgaris, pemphigus vegetans, pemphigus foliaceus, pemphigus erythematosus, paraneoplastic pemphigus (PNP), and drug-related pemphigus are the main variations of the disease. Over 80% of cases of pemphigus are Pemphigus Vulgaris, making it the most prevalent type.3
IgG autoantibodies that attach to keratinocyte cell surface molecules are linked to blisters. PV antibodies attach to desmosome-free regions of the keratinocyte cell membrane as well as to desmosomes in keratinocytes.4 Anti-inflammatory drugs, drugs that decrease the production of antibodies, and procedures that improve antibody elimination are only a few of the therapy options available for this disorder.5 The purpose of this case study is to describe a typical instance of this ailment and to draw attention to the various therapeutic choices available.
Case Report
A 59-year-old female patient reported with the chief complaint of ulcers in the mouth and difficulty in swallowing food since 1 year. Patient first noticed difficulty for swallowing solid food and liquid which progressively increased in severity. The patient had noticed ulcers of the mouth which bleed on brushing. The patient did not report any skin lesions or involvement of other mucosal sites. A review of medical and family history was noncontributory.
On extra oral examination, [Figure 1] right and left submandibular lymph nodes were enlarged, palpable and tender. Intra-oral examination revealed multiple ulcers present on upper and lower labial mucosa, right and left buccal mucosa, palate, tongue.[Figure 2, Figure 3, Figure 4] Ulcers presented with the well-defined borders with yellowish slough with the areas of reddish erythema. Ulcers were tender on palpation and not attached to the underlying structures. Based on the clinical presentation, provisional diagnosis of pemphigus vulgaris was given.
Incisional biopsy on left buccal mucosa was carried out and report revealed presence of epithelium and connective tissue. The epithelium is parakeratinized stratified squamous type showing acantholysis. A characteristic suprabasilar split with hemorrhagic spots and acantholytic cells known as Tzank cells are seen. Areas revealing basilar tombstones are present. Subepithelial connective tissue shows chronic inflammatory cell infiltration of lymphocytes and numerous adipocytes. Few constricted blood vessels engorged with RBCs are seen. Deeper stroma shows both longitudinal and transverse sections of muscle bundles. Correlating with the given clinical features, the above 'histopathological features are suggestive of Pemphigus.
Treatment is usually targeted at controlling the severity and preventing relapses. Systemic corticosteroids remain the gold standard treatment for pemphigus. Patient was treated with topical Triamcinolone acetonide 0.1%, applied 3 times daily for oral lesions.
Discussion
Pemphigus is a category of potentially fatal blistering skin and mucous membrane disorders distinguished by acantholysis (loss of keratinocyte to keratonicyte adhesion).3
The Greek word "Pemphix," which means bubble, is the source of the term "pemphigus." A set of rare but potentially fatal autoimmune diseases known as pemphigus vulgaris are characterised by mucosal and cutaneous blisters. Epithelial blistering that develops from erythematous macules or urticarial bases is its defining feature.6
Wichman initially coined the term "pemphigus" in 1791 to refer to all blistering disorders collectively (Korman 1988). In 1953, Lever distinguished PV from bullous pemphigoid based on the histological image, clinical criteria, and progress of the disease.7
Desmoglein 3 (Dsg3) and desmoglein 1 (Dsg1) are desmosomal cadherins that Pemphigus targets, but Pemphigus foliaceus solely targets Dsg1. The distribution of blistering is influenced by the distinct expression patterns that Dsg3 and Dsg1 exhibit in mucosa and skin. Dsg1 is expressed significantly less and mostly in the superficial layers of the mucosa, but Dsg3 is expressed in much higher numbers throughout the entire epithelium. While Dsg1 is distributed across the entire epidermis and particularly strongly in the superficial layers, Dsg3 is extremely weakly expressed in skin and is only found in the basal and parabasal layers.8
People commonly develop pemphigus between the ages of 40 and 50, with femaless allegedly being affected more commonly than men. More than 50% of the patients affected have been observed to have oral mucosal symptoms at first, followed by skin involvement. It is discovered that oral lesions last, on average, between three months and a year.9
Pemphigus vulgaris oral symptoms can appear either beforehand or concurrently with cutaneous lesions.10 Pressure or friction can cause blisters to form on normally looking skin or mucosa (Nikolsky sign). Clinically, the lesion presents as thin-roofed, short-lived blisters that are brittle and prone to rupture, leading to painful and hemorrhagic erosions. In our case, the disease first showed symptoms as oral lesions of the gingiva and palate.11 The buccal mucosa is the most frequently affected area of the oral mucosa, followed by the palatal, lingual, and labial mucosa. Lesions can develop anywhere on the oral mucosa. The gingiva is least frequently affected, and desquamative gingivitis is the most typical symptom of the condition. In many patients, oral lesions are followed by the development of skin lesions.3
Early diagnosis, when lower dosages of medication can be administered for shorter periods of time to control the disease, is a crucial component of patient therapy. Topical and systemic corticosteroids are currently used in combination with immunosuppressants like azathioprine or mycophenolate to treat pemphigus vulgaris. Intravenous immunoglobulin or plasmapheresis, which work to lower levels of circulating autoantibodies, can also be added to the treatment. Short-term disease remission has been demonstrated to be effectively induced by anti-CD20 antibody rituximab therapy. After the lesions have disappeared, a maintenance regimen is required to keep the disease under control and lessen the negative effects of the medications. Depending on how much the lesion is involved, the length of medical treatment varies.10
A lengthy clinical course and considerable morbidity and death are associated with pemphigus vulgaris. If pemphigus is not treated, mortality increases by 50% after two years and reaches almost 100% after five years. Significant skin involvement, septicemia, bronchopneumonia, electrolyte imbalance, and subsequent systemic infections were the main reasons of death.10
Conclusion
In this investigation, PV was the most common subtype of pemphigus. A common but potentially fatal autoimmune mucocutaneous condition, pemphigus vulgaris has a propensity to attack the oral mucosa. Although the exact cause of PV is unknown, a number of diverse elements may contribute to its pathogenicity. Most PV cases started off with mouth lesions. The clinician typically encounters the oral symptoms of pemphigus vulgaris first and occasionally as the only symptom. Timely diagnosis and prompt treatment are hence necessary for complete cure with reduced morbidity and mortality owing to the chronic and often fatal course of this condition.
