case_images: PMC8560286_01
case_images: Mucosal damage affecting the entire oral cavity. The lesions were located in the internal surfaces of the lips and cheeks, the dorsal surface of the tongue, and the palatal fibromucosa. There were multiple epithelial erosions and postbullous ulcerations.
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case_images: fig1
case_images: 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).
case_images: Blisters of the oral mucosa with a positive Nikolsky sign. Presence of poor oral hygiene-associated bullous lesions and ulcerations. Nikolsky sign: epithelial cells are separated from the basement membrane, allowing an assessment of the fragility of epithelial attachment mechanisms (acantholysis).
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case_images: fig2
case_images: There were epithelial erosions and postbullous ulcerations with a positive Nikolsky sign indicating acantholysis with dislocation of the epithelial layers (Figure 2).
case_images: Aspect of the umbilical lesions. Erosions and ulcerations with erythema affecting the umbilical area associated with Staphylococcus aureus growth.
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case_images: fig3
case_images: Bulloerosive umbilical involvement was associated with the oral impairment without noting any other cutaneous lesions for four months (Figure 3).
case_images: Worsening of the lesions 5 days after the first consultation. Extension of the lesions with appearance of multiple crusts in the lips and umbilicus.
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case_images: fig4
case_images: In a while, her condition worsened (Figure 4).
case_images: Histopathological and IFD of the oral biopsy. 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.
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case_images: Direct immunofluorescent examination was negative (Figure 5).
case_images: Histopathological and IFD of the skin 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.
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case_images: fig6
case_images: Intercellular immunoglobulin G and C3 deposits were observed on direct immunofluorescent examination with a characteristic mesh-like appearance (Figure 6).