Mucosal-dominant pemphigus vulgaris in a 90 Y/O/F: Case repo
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A 90 year old Japanese woman was referred with a 3-month history of painful gingivae that first appeared at age 90 years. Patient reported difficulty in eating because of multiple painful erosions following easily ruptured vesicles in the buccal mucosa and tongue during the last 3 weeks. Medical history included hyperlipidaemia and reflux esophagitis, for which she regularly consumed atorvastatin calcium hydrate and esomeprazole.

A general examination revealed a few erythematous erosions on the back. An intraoral examination revealed multiple erosions and desquamation with erythematous areas on the right and left buccal mucosa, palate, lateral border of the tongue and gingiva. She was positive for Nikolsky’s sign.

A gingival biopsy for histopathology revealed intraepithelial cleavage with acantholysis in the suprabasal region and retaining of basal keratinocytes along the basement membrane zone. Direct immunofluorescence (IF) study showed the deposition of IgG and C3 between the epithelial cells . The indirect IF ELISA for desmoglein-3 and desmoglein-1 antibodies revealed their elevated levels. Based on all clinical, histopathological and serological findings, we confirmed mucosal-dominant PV with minimal skin lesions was diagnosed.

The patient received oral prednisolone at an initial dosage of 35 mg/day (1 mg/kg/day). This helped in rapid epithelialisation of the erosions on the back. The intraoral erosions diminished significantly in 2 weeks, and the dose was tapered to 30 mg/day. A marked improvement in the lesions was observed on subsequent follow-up. The patient is currently on prednisolone treatment (15 mg/day) and to date no other lesions have been reported.

Early diagnosis is challenging since the oral lesions may be relatively non-specific, emerging as superficial erosions or ulcerations and rarely manifesting as intact bullae. Physicians should be sufficiently familiar with the oral manifestations of PV, such as gingivitis, even in the oldest older patients, and should involve specialists to ensure early diagnosis and treatment, thereby influencing the prognosis and course of this disorder.

Source: https://casereports.bmj.com/content/13/10/e238586?rss=1
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