Pemphigus Vulgaris

Pemphigus Vulgaris (‘vulgaris’ is Latin for ‘common’) is a vesiculo bullous autoimmune disease of unknown cause that affects the skin and mucous membranes. The autoantibodies are directed against the epidermal cell surface glycoproteins desmogleins which are components of desmosomes, the structure that bonds epithelial cells to each other. The autoantibodies activate the complement system and mediate for the loss of cell-to-cell adhesions (acantholysis) resulting in blister formation. [1]

The initial manifestations of pemphigus vulgaris often involve the oral mucosa, typically in adults. The average age at diagnosis is 50 years. Although rare cases may be seen in childhood. No sex predilection is observed, and the condition seems to be more common in Jews. Patients usually complain of oral soreness. and examination shows superficial. ragged erosions and ulcerations distributed haphazardly on the oral mucosa. Such lesions may affect virtually any oral mucosal location. Although the palate labial mucosa. buccal mucosa, ventral tongue and gingivae are often involved. Patients rarely report vesicle or bulla formation intraorally, and such lesions can seldom be identified by the examining clinician probably because of early rupture of the thin friable roof of the blisters. Over 50% of the patients have oral mucosal lesions before the onset of cutaneous lesions. Sometimes by as much as I year or more. Eventually, however, nearly all patients have intraoral involvement. The skin lesions appear as flaccid vesicles and bullae that rupture quickly. Usually within hours to "few days”, leaving an erythematous, denuded surface. In frequently ocular involvement may be seen, usually appearing as bilateral conjunctivitis. Unlike cicatricial pemphigoid, the ocular lesions of pemphigus do not tend to produce scarring and symblepharon formation. [2-4]

Without proper treatment, the oral and cutaneous lesions tend to persist and progressively involve more surface area. A characteristic feature of pemphigus vulgaris is that a bulla" can be induced on normal-appearing skin if firm lateral pressure is exerted. This is called a positive Nikolsky sign. [3]

Pemphigoid

Pemphigoid is seen as a family of disease that include conditions like Bullous Pemphigoid (BP), pemphigoid (herpes) gestationis which generally affect the skin and have only minor oral  involvement and Mucous  Membrane. Pemphigoid (MMP) mainly involving the mucous membranes, most frequently the oral and ocular mucosa. [5]

MMP is a disease of late middle to old age and more common in women. In 1987, Gallagher and Shklar reported a study of 120 patients of women (87.5% Vs. 121.5% men) The age of onset was between 40 – 49 years for 25% of the patients, between 50 – 59 years for 31.6% and between 60 – 69 years for 22.5%.

There is no ethnic or geographic predilection. The striking features are the recurring blisters on either a mucous membrane or an area of skin together with the tendency for scarring. The initial site may be any mucous membrane like conjunctiva or oral mucosa but nose, larynx, pharynx, esophagus, genital mucosa are also involved. [6]

The oral cavity is the most common site for CP accounting for 83-100%. In Gallagher & Shklar study [6] all (100%) the patients had gingival lesions. Oral lesions of MMP most commonly manifest as desquamative gingivitis of the marginal and attached gingiva [6]. Patients often present with the alveolar mucosa. Desquamation results from exposure of the oral mucosa to inflammation and trauma from mastication and presents as white areas of necrotic slough at the margins of the erythematous zones and may be elicited by palpation. [7]

Vesicles or bullae may occur on hard and soft palate, buccal mucosa, alveolar ridge and tongue and there can be a positive Nikolsky sign. The blisters rupture quickly leading to pseudomembrane covered, irregularly shaped erosions having a yellowish slough surrounded by an inflammatory halo. Lip lesions are uncommon. A significant feature of the oral lesions is their ability to heal without scarring. [7-9]

Systemic Lupus Erythematosus (SLE) and Discoid Lupus Erythematosus (DLE)

Systemic Lupus Erythematosus (SLE) is a chronic generalized inflammatory connective tissue disease with characteristic autoantibodies primarily affecting the skin, musculoskeletal system, serous membranes, kidneys, central nervous system (CNS) and cells of the blood. Also, it is considered the most common systemic autoimmune connective tissue disorder. Lupus erythematosus was first described in 1828 by Biett. [10]

SLE has been sub-classified into various subsets though it is not known whether the variations in clinical expression is due to variations in host responsiveness to a single etiological stimulus or whether different patterns of disease. However sub-classification of disease has led to the realization that certain antibodies appear to be associated with particular pattern of illness (eg. Anti-Ro with cutaneous disease) and also has allowed the designation of prognosis to each variant. [11]

Systemic lupus erythematosus (SLE) is a syndrome characterized by a broad spectrum of manifestation involving skin, joints, kidneys, lungs, nervous system, serosal cavities, hematopoietic system or other organs of the body. The diagnostic criteria of SLE are defined according to the preliminary criteria of the American Rheumatism Association (ARA). [11]

Discoid LE (DLE) is a chronic disease mainly limited to the skin and / or mucous membranes and the prognosis is good. Only 10% to 20% of patients with DLE develop systemic manifestations later in the course of the disease.

The overall prevalence of SLE is 0.05% with an incidence between 1.8 to 7.6 cases per 100,000 per year in the United States. Lupus is more common in Asians, African Americans, African Carribeans and Hispanic Americans than in whites. Prevalence of oral DLE was 0.01%.

Familial cases occur in about 10%, relatives of patients with SLE have an increased incidence of SLE & DLE, rheumatoid arthritis, rheumatic fever, polyarteritis nodosa, dermatomyositis. [12-13]

SLE occurs in early adult life and peak age of onset is 38 years in females and 44.2 in men. Onset of oral discoid lesions may occur at any age (range 6–77 years). SLE is more frequently found in women, with a female to male ratio of 8:1 in adults. The female to male ratio is much smaller in children and in elderly persons dropping to 2:1. [14-15]

DLE 

Reported prevalence of oral lesions in patients with DLE in skin was about 20% by Andreasan 1964 and 25% by Shklar & Mccarthy 1978. The prevalence of skin lesions among 66 patients with oral discoid lesions was 56%. The predilection sites are with decreasing frequency, the buccal mucosa, gingiva, labial mucosa and vermilion border. Oral discoid lesions are long standing chronic lesions and about 75% of the patients experience burning sensation from hot and spicy food. It clinically appears with a central atrophic red area with small white dots and a slightly elevated border zone of irradiating white striae and telangiectasia. The lesions are infected by yeasts in half of the cases.

Considerable clinical variations may occur. The vermilion border lesions show scaling and crusting while lesions on attached mucosa such as gingiva and hard palate often lack the irradiating white striae. Early lesions may show erythema only however with years they may turn to leukoplakia like lesions.

SLE 

The oro pharyngeal or oral ulceration is one of the ARA – criteria and occurred in 8–40% of ARA classified SLE patients in American & Mexican patients .The predilection site for erythematous lesions and ulcerations is the hard palate. The oral lesions may persist for several years but can show remission and exacerbation and be present intermittently. Clinically erythematous lesions, discoid lesions and ulcerations, may merge into another. Of these ulcerations dominate during acute systemic disease. Other oral manifestations associated with SLE and DLE. [16-18]

Infections by yeasts on oral lesions occur especially during exacerbation of disease and / or during systemic steroid treatment [18]. Herpes simplex and herpes zoster infections also occur in SLE. In patients with co-existent SLE & Sjogren’s syndrome xerostomia and salivary gland enlargement can be seen .Bone involvement is rare in SLE and arthralgia and arthritis in SLE is non-deforming. Clinical dysfunction of TMJ was reported in 41% of SLE patients showing radiographic changes in 30%, which were flattening, and erosion of the mandibular condyle.

Conflict of Interest

None declared

Funding

No funding sources

Ethical Approval

The study was approved by the Institutional Ethics Committee of H.P Govt Dental College  Hospital

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