International Journal of Nutrition, Pharmacology, Neurological Diseases

CASE REPORT
Year
: 2013  |  Volume : 3  |  Issue : 2  |  Page : 146--149

Pemphigus vulgaris of oral cavity: A case report with its treatment strategies


Seema Kapoor1, Pranav Sikka2, Geet Priya Kaur1,  
1 Department of Oral and Maxillofacial Pathology, ITS-CDSR (Muradnagar), Ghaziabad, Uttar Pradesh, India
2 Department of Pharmacology, Lala Lajpat Rai Memorial Medical College, Meerut, Uttar Pradesh, India

Correspondence Address:
Seema Kapoor
Department of Oral and Maxillofacial Pathology, ITS-CDSR (Muradnagar), Ghaziabad, Uttar Pradesh
India

Abstract

Pemphigus vulgaris (PV), a rare autoimmune mucocutaneous blistering disorder, has been reported with incidence of 0.1-0.5 cases per 100,000 individuals worldwide per year. It is slightly more common in women and occurs primarily in adults during the 5 th or 6 th decade of life. The etiology of PV is uncertain but is supposed to be mediated by circulating immunoglobulin G (IgG) autoantibodies against the desmosomal cadherins, desmoglein 1 and 3. Biopsy, light microscopic examination, and additional adjuvant tests, such as immunofluorescence studies can be used to establish the diagnosis. In most cases (70-90%), the first signs of disease appear on the oral mucosa but most patients with oral lesions are initially misdiagnosed and treated improperly for months or years. If these patients are misdiagnosed or left untreated, PV may be fatal with a mortality rate ranging from 60% to 90%. Therefore, here we are trying to discuss basics of diagnosing and treating PV with oral lesions with the help of a case report.



How to cite this article:
Kapoor S, Sikka P, Kaur GP. Pemphigus vulgaris of oral cavity: A case report with its treatment strategies.Int J Nutr Pharmacol Neurol Dis 2013;3:146-149


How to cite this URL:
Kapoor S, Sikka P, Kaur GP. Pemphigus vulgaris of oral cavity: A case report with its treatment strategies. Int J Nutr Pharmacol Neurol Dis [serial online] 2013 [cited 2020 Oct 30 ];3:146-149
Available from: https://www.ijnpnd.com/text.asp?2013/3/2/146/112842


Full Text

 Introduction



Pemphigus represents a group of rare autoimmune mucocutaneous blistering disorders. The two main subtypes are pemphigus vulgaris (PV) and pemphigus foliaceous, each with its own clinical variants. Pemphigus vulgaris is a rare disorder, with a reported incidence of 0.1-0.5 cases per 100,000 individuals worldwide per year. It is slightly more common in women and occurs primarily in adults during the 5 th or 6 th decade of life. [1]

The disease is mediated by circulating immunoglobulin G (IgG) autoantibodies against the desmosomal cadherins, desmoglein 1 and 3. Histopathology reveals a loss of cell-cell adhesion (acantholysis) in the suprabasilar layer of the epithelium, and direct immunofluorescence (DIF) of perilesional skin reveals intercellular deposition of IgG +/- C3. As antibodies often correlate with disease activity, indirect immunofluorescence (IIF), immunoblots, and enzyme-linked immunosorbent assays (ELISA) are commonly used to quantify circulating antibody levels. [2]

To make the diagnosis of pemphigus vulgaris, the clinician needs to perform a biopsy of the lesional tissue. [3] If left untreated, PV is frequently fatal with a mortality rate ranging from 60% to 90%. [4]

 Case Report



A 31-year-old male presented with chief complaint of difficulty in eating food due to ulcers in the mouth. The ulcers were present for 7-8 months. There was sore throat at first followed by ulcers all over mouth, which healed incompletely in 2½ months after medication by local practitioner, but the ulcers recurred after stoppage of medication.

The patient had no history of tobacco or any other addiction. There was no history of long-term treatment for any chronic illness or continuous drug intake. No significant family history was found.

There was no noticeable finding on general examination. On physical examination, a few ruptured vesicles, crusted plaques, and pigmented macules were found over the trunk. On intra-oral examination, multiple diffuse erosive lesions over buccal mucosa were found bilaterally with erythematous areas over anterior gingiva. [Figure 1]a-c Tenderness was present on palpation. Incisional biopsy from the representative site was performed under local anesthesia (LA) with due consent of the patient.{Figure 1}

The cytological smear was prepared, which showed clusters of darkly stained, round acantholytic cells. [Figure 2] Histopathology revealed an area of normal stratified squamous epithelium, denuded epithelium, and an intraepiththelial clefting with hyperchromatic acantholytic cells along with chronic inflammatory infiltrate in the connective tissue. [Figure 3] and [Figure 4]{Figure 2}{Figure 3}{Figure 4}

The patient was diagnosed to have PV and was prescribed systemic prednisone (80 mg/day in divided doses per oral) with local anesthetic gel application for symptomatic relief. He was also given an antibiotic (doxycycline 100 mg bid per oral) and antiseptic (povidone iodine) gargles. Later, Cyclophosphamide (1 mg/kg/day) was added as steroid-sparing agent. The patient responded positively and prednisone was tapered gradually up to 10 mg/kg/day. The patient is still in follow-up and is doing well.

 Discussion



PV is characterized by intraepithelial blister formation that results from breakdown of cellular adhesion between epithelial cells. In most cases (70- 90%), the initial symptoms of disease appear on the oral mucosa. [1] The oral lesions may be painful and may interfere with eating or other oral functions. The most common sites of oral involvement include the buccal mucosa, soft palate, labial mucosa, and gingiva, although any oral site may be affected. [3] The etiology of PV is uncertain as is with other auto-immune diseases. [5],[6] In some cases, it is found to have a strong genetic basis, as it has been reported in certain racial groups, for example, the Ashkenazi Jews and those of Mediterranean descent. Strong association between certain HLA class II alleles has also been demonstrated. Other initiating factors include certain foods, infection, neoplasms, and drugs. The drugs commonly implicated are those in the thiol group, in particular captopril, pencillamine, and others such as rifampicin. [4] Most patients with oral lesions could be initially misdiagnosed, usually as apthous stomatitis, gingivostomatitis, erythema multiforme, erosive lichen planus, or oral candidiasis, and may be improperly treated for months or years. Other differential diagnoses include dermatitis herpetiformis and cicatricial pemphigoid. [1]

Biopsy, light microscopic examination, and additional adjuvant tests, such as IIF studies, can be used to establish the diagnosis. The above investigations will show the production of antibodies directed against desmosomal components resulting in separation of epithelial cells above the basal cell layer. [3]

Systemic corticosteroids remain the treatment of choice for pemphigus as they are both effective and capable of inducing a rapid remission. However, adverse effects of corticosteroids are both time- and dose-dependent. They include weight gain, diabetes, hypertension, glaucoma, cataract, osteoporosis, avascular bone necrosis, peptic ulcer disease, adrenal insufficiency, electrolyte and lipid abnormalities, psychosis, immune-suppression, and increased susceptibility to infections. [7] Adjuvant therapies are, therefore, used to provide a steroid-sparing effect. As these treatments typically have a slower onset of action, viz., 4-6 weeks, they are most beneficial as maintenance therapies. Conventional adjuvants include various immunosuppressive agents such as azathioprine, mycophenolate mofetil, methotrexate, cyclophosphamide, chlorambucil, and cyclopsorine, as well as anti-inflammatory agents such as gold, dapsone, colchicines, and a variety of tetracycline antibiotics. [8],[9],[10] Unfortunately, these medications are often associated with significant toxicities and must be used with caution. Although the majority of patients will ultimately respond to conventional therapies, a few patients develop recalcitrant disease. Emerging therapies include intravenous immunoglobulins, plasmapheresis, immunoadsorption (IA), extracorporeal photochemotherapy (ECP), monoclonal antibodies such as rituximab, tumor necrosis factor-alpha (TNF-α) antagonists, viz., infliximab and etanercept, cholinergic agonists, and other experimental therapies such as desmoglein 3 peptides and KC706. [2] Some studies have suggested Co-enzyme Q10 as adjuvant therapy for periodontal involvement in PV. [11]

If undiagnosed, PV may lead to grave consequences. The article focuses on timely diagnosis and appropriate management of a pemphigus patient. Therefore, we need to induce and maintain remission with the lowest possible doses of medication, so as to minimize the risk of serious and potentially fatal adverse effects.

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