PMC2913839_01
Blisters and erosions on the buccal mucosa, tongue, and lips. Oral lesions did not extend onto the vermillion of the lips.
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On examination, he had blisters and erosions on the buccal mucosa, tongue and lips (Figure 1).
Scattered crusted and erosive lesions with flaccid bullae on the chest.
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He also had several scattered crusted and erosive lesions with small flaccid bullae on the chest and back (Figure 2).
Histological findings of the skin lesion. Subcorneal bulla with mild acantholysis. No interface dermatitis was seen.
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Biopsy of the flaccid bulla on the chest showed subcorneal bulla formation with mild acantholysis (Figure 3).
Histological findings of the oral lesion. Severe lichenoid dermatitis with dyskeratotic cells.
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Histopathological findings of the oral mucosa revealed severe lichenoid dermatitis with dyskeratotic cells (Figure 4).
Direct immunofluorescence of the perilesional skin. Intercellular deposits of IgG throughout epidermis.
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Direct immunofluorescence of the perilesional skin showed intercellular deposits of IgG throughout epidermis and intercellular deposits of C3 in the lower layer of epidermis (Figure 5).
Direct immunofluorescence of the oral mucosa. Cell surface deposits of IgG in the lower layer of epithelium with ovoid bodies.
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IgG and IgM (Figure 6). Indirect immunofluorescence (IIF) revealed anticell surface antibodies at a titer of 1:160 with normal human skin. IIF was also positive using monkey esophagus and rat bladder epithelium as a substrate (Figure 7).
Indirect immunofluorescence was positive using rat bladder epithelium as a substrate.
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IgG and IgM (Figure 6). Indirect immunofluorescence (IIF) revealed anticell surface antibodies at a titer of 1:160 with normal human skin. IIF was also positive using monkey esophagus and rat bladder epithelium as a substrate (Figure 7).
Results of immunoblot analysis using epidermal extract. Patient's sera taken on 2 different occasions (lanes 4 and 5, the time interval was 26 days) recognized BP230 and 190 kDa periplakin, which were also detected by control bullous pemphigoid and control PNP sera. No reaction with 210 kDa envoplakin was observed. Lane 1: pemphigus vulgaris control serum, Lane 2: PNP control serum, and Lane 3: bullous pemphigoid control serum. The serum dilution was 1:20.
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By immunoblot analysis using normal human epidermal extract, the patient's serum reacted with BP230 and the 190 kDa periplakin, but not with the 210 kDa envoplakin (Figure 8).
Results of immunoprecipitation using radiolabeled cultured keratinocytes. Control PNP serum (PNP) immunoprecipitated the 250 kDa desmoplakin I, BP230, the 215 kDa desmoplakin II, the 210 kDa envoplakin, the 190 kDa periplakin, and the 170 kDa unknown PNP antigen (lane 3). The serum of this patient (Patient) immunoprecipitated desmoplakin I, BP230 and envoplakin (lane 2). Normal control serum (Normal) showed no positive reaction (lane 1).
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In contrast, by immunoprecipitation using radiolabeled cultured keratinocytes, the serum of this patient immunoprecipitated the 250 kDa desmoplakin I, BP230 and envoplakin, but not the 215 kDa desmoplakin II, periplakin and the 170 kDa unknown PNP antigen (Figure 9).