A 55-year-old woman with no notable medical and family history was referred to our department by her general practitioner following the appearance of multiple lesions of the oral mucosa. The intraoral clinical examination revealed poor oral hygiene, hypersialorrhea, and extensive mucosal damage affecting the entire oral cavity and causing discomfort, pain, and dysphagia. The lesions were located in the internal surfaces of the lips and cheeks, the dorsal and ventral surface of the tongue, and the soft palate mucosa (Figure 1). There were epithelial erosions and postbullous ulcerations with a positive Nikolsky sign indicating acantholysis with dislocation of the epithelial layers (Figure 2). Bulloerosive umbilical involvement was associated with the oral impairment without noting any other cutaneous lesions for four months (Figure 3).
In front of these signs, we conducted an oral biopsy and prescribed topical prednisolone (20 mg; 3 times per day) and chlorhexidine-based (0.12%; 3 times per day) mouthwashes in order to reduce the oral pain awaiting the biopsy result. In a while, her condition worsened (Figure 4). We referred her to the department of dermatology where she was hospitalized and an umbilical biopsy was performed.
On histopathological examinations, the oral biopsy concerned a squamous mucosa lined with a partially visualized coating, with disappearance of the superficial layers and the presence of cells of the basal layer which remain attached to the underlying chorion, drawing a "tapestry nail" appearance, surmounted by a few acantholytic cells. In the chorion, we noted the presence of a polymorphic inflammatory infiltrate rich in eosinophilic polynuclear cells. Direct immunofluorescent examination was negative (Figure 5). As for the umbilical biopsy, the sample received was carried to the deep dermis. It was bordered by the basal layer of the epidermis, drawing the same "tapestry nail" appearance with extensive suprabasal acantholysis and separation of the overlying epidermal layers. The dermis was the site of an inflammatory infiltrate rich in neutrophils and eosinophils of perivascular arrangement with extravasation of red blood cells and without alteration of their wall. Intercellular immunoglobulin G and C3 deposits were observed on direct immunofluorescent examination with a characteristic mesh-like appearance (Figure 6). Based on clinical, histopathological, and immunofluorescence findings, a diagnosis of pemphigus vulgaris (PV) was established.
On laboratory investigations, we found an inflammatory syndrome with increased erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). Hepatitis (VHB and VHC) and VIH serologies were negative. Mycological direct examination and cultures were negative. On bacteriological culture performed from the umbilicus, Staphylococcus aureus growth was observed.
As for the therapeutic management, due to oral pain, a nasoenteral probe was used to feed her. Daily intravenous administration of prednisolone (120 mg/day) and amoxicillin-clavulanic acid (1000 mg thrice a day) was started. The nursing team was instructed to clean the oral cavity and the umbilicus using chlorhexidine (0.12%) mouthwash and ointment. Her condition improved gradually during hospitalization. There was a complete remission of the lesions in the mouth and umbilicus. The patient was advised to continue oral prednisone intake at a dose of 20 mg/day. She underwent monthly follow-ups in dentistry and dermatology departments after discharge from the hospital.
Four months later, the disease recurred. We noticed the appearance of severe cutaneous lesions added to the oral and umbilical ones located in the elbows, forearms, and the periungual area. She was newly hospitalized.