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Pemphigus is a group of autoimmune diseases characterized by formation of intraepithelial bullae in skin and the mucous membrane. Pemphigus vulgaris affects the oral mucosa in nearly all cases. Pemphigus vulgaris is characterized by auto antibodies directed against desmosome-associated protein antigens (desmoglein-3) found in epithelial and epidermal intercellular substance. We report here a case of pemphigus vulgaris of gingiva in an adult female patient at an early stage followed by dermatologic involvement. Perilesional incision was taken and histopathological and direct immunofluorescence was done for identification of specific antibodies. The oral lesions were treated with 0.1% Triamcinolone acetonide ointment and Prednisolone 20 mg twice daily with multivitamins was administered systemically for skin lesion.
A number of dermatological diseases may present gingival manifestations sometimes in the form of desquamative lesions of the gingiva or gingival ulcerations. Desquamative gingivitis (DG) denotes a particular clinical picture and is not a diagnosis in itself. The common disease with relevance of these diseases include Lichen planus, Pemphigoid, Pemphigus, Erythema multiforme, and Lupus erythematosus.
Pemphigus is a group of autoimmune diseases characterized by formation of intraepithelial bullae in skin and the mucous membrane. Pemphigus vulgaris is the most common of pemphigus which also includes pemphigus foliaceus, pemphigus vegetans, and pemphigus erythematosus. Pemphigus is a rare case of desquamative gingivitis, one study showed that only 2.3% of DG is due to pemphigus. Studies have shown world-wide incidence of 0.1 to 0.5 cases of pemphigus vulgaris per year per 1lakh individuals.[2,4] Pemphigus vulgaris affects the oral mucosa in nearly all cases and more importantly, the oral mucosa is the site of the first lesion in the majority of cases. Pemphigus vulgaris is characterized by auto antibodies directed against desmosome associated protein antigens (desmoglein-3) found in epithelial and epidermal intercellular substance. Since the desmosome is the primary attachment mechanism between keratinocytes, the inflammatory destruction of that attachment leads to the characteristic fluid filled bullae and subsequent ulceration. We report here a case of pemphigus vulgaris of gingiva in an adult female patient at an early stage followed by dermatologic involvement.
A 36-year-old married female [Figure 1], resident of Pune, housewife by occupation referred by Department of Dermatology, Command Hospital Pune for the gingival lesion to division of Periodontics, Department of dental surgery, AFMC, Pune, with chief complaint of burning sensation of the oral cavity for the last 3 months. The patient was apparently asymptomatic before 3 months. She noticed ulcers in the mouth [Figure 2] and felt severe burning sensation, specially aggravated on taking hot and spicy food. The vesicles on the gum ruptured to produce pain and severe itching in the mouth. She also noticed small eruptions over skin on face, back [Figures [Figures33 and and4]4] extremities just 3 weeks before, for which she reported to the dermatologist.
The medical history and family history was clear and patient was not taking any other drugs and had no ocular or genital lesions. The patient had a proper oral hygiene habit without any deleterious habits.
On general physical examination, the patient was moderately built and nourished with vital signs within normal limits. She had numerous flaccid vesicles and ruptured bullae of varying diameter seen over the back and nose, which are mainly seen as ulcerated eroded surfaces. On extra oral examination no relevant findings were observed.
On intra-oral examination, denuded and spontaneously bleeding gingival zones were revealed. The clinical picture of persistent superficial ulcers with a positive Nikolsky's sign strongly suggested a vesicullobulous disorder. Based on the history, clinical signs, and symptoms of both intaoral and extraoral lesions, a provisional diagnosis of desquamative gingivitis was given.
After routine hemogram, urine and blood sugar examination of patient was taken up for incisional perilesional biopsy with intact epithelium. The sample was sent for histopathological examination in normal saline to Department of Pathology.
The patient was taken back to Department of Dermatology for opinion and biopsy of skin was taken from back for histopathological examination and for the direct immunofluorescence assay in Michel's solution. On histopathological examination, acantholysis was revealed along areas of ulceration without dysplastic changes with polymorphonuclear leucocytes infiltration. Basal cells of epithelium remained attached to the basement membrane. Higher resolution showed perivesicular edema with loss of cohesiveness and hyperchromatic epithelial cells called “Tzank cells” [Figures [Figures55 and and6],6], suggesting diagnosis of pemphigus vulgaris. The direct immunofluorescence showed intercellular deposition of C3, IgG, and IgM in stratum spinosum [Figures [Figures77 and and8]8] confirming the diagnosis of pemphigus vulgaris.
The oral lesions were treated with 0.1% Triamcinolone acetonide ointment and skin lesion with topical application of 0.05% Clobetasol with Soframycin twice daily. Prednisolone 20_mg twice daily with multivitamins was administered systemically. The patient is under control and still under review [Figure 9].
It is reported that most cases of DG are cases of severe mucocutaneous diseases.[3,5,6] In the present case report of pemphigus vulgaris, the oral lesions were the first sign of the disease with subsequent dermatologic involvement as observed by several other studies.[7,8] Mucous membrane pemphigoid and erosive lichen planus are the most common causes of DG accounting for 48.9% and 23.6% respectively, pemphigus vulgaris being the least common cause.
Pemphigus vulgaris is potentially fatal considering the nature of the disease. It is essential to establish a early definitive diagnosis, timely therapy and follow up. The present case under discussion has visited various dental clinics for her oral lesions, when she had not developed the skin lesion. But definitive diagnosis was not rendered at any level. The reasons for delayed diagnosis may be explained by the patient symptoms being confined to gingiva and clinically mild in nature and the sign and symptoms occurring in repeated cycles of remission and exacerbation. In this report, we recognized the acantholytic cells in the histopathologic study which was not sufficient for a confirmed diagnosis of pemphigus vulgaris. The confirmed diagnosis was made after the direct immunofluorescence assay method was followed from the skin lesion. This is because acantholytic cells may also appear in Impetigo, Darier's disease, transient acantholytic dermatosis, viral infections, and carcinoma.
To summarize, in the present case, a definitive diagnosis of pemphigus vulgaris was made based on a general assessment of positive Nikosky's sign, presence of acantholysis in histopathology and findings of direct immunofluorescence.
High dose topical corticosteroid ointment may control limited oral disease. In most cases, however, disease control or remission is achieved using systemic corticosteroids alone or in combination with immuno-modulating medications. The most important aspect of pemphigus vulgaris is its early recognition, diagnosis, and treatment. The dentist has a unique opportunity to make the diagnosis and institute treatment.
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Conflict of Interest: None declared.