Paraneoplastic pemphigus presenting as mild cutaneous features of pemphigus foliaceus and lichenoid stomatitis with antidesmoglein 1 antibodies

Abstract

Herein, we report a case of paraneoplastic pemphigus with mild skin features of pemphigus foliaceus and lichenoid stomatitis associated with B-cell lymphoma. A 49-year-old man presented with scattered blisters and erosions on the trunk along with mucosal blisters and erosions. Skin biopsy showed subcorneal acantholytic bulla and oral mucosal biopsy demonstrated lichenoid dermatitis. Direct immunofluorescence showed cell surface deposits of IgG and C3. Indirect immunofluorescence identified circulating IgG autoantibodies to the cell surfaces of normal human skin and also on the transitional epithelium of rat bladder. Enzyme-linked immunosorbent assay using recombinant baculoproteins showed positive antidesmoglein 1 autoantibodies (index 46) but negative antidesmoglein 3 autoantibodies (index 8). Immunoblot analysis using normal human epidermal extract detected BP230 and the 190 kDa periplakin, while immunoprecipitation using radiolabeled cultured keratinocyte immunoprecipitated BP230 and the 210 kDa envoplakin. We consider that the skin lesion was produced by humoral immunity whereas the oral lesion was produced by cellular immunity.

Figure 1

Blisters and erosions on the buccal mucosa, tongue, and lips. Oral lesions did not extend onto the vermillion of the lips.

Figure 2

Scattered crusted and erosive lesions with flaccid bullae on the chest.

Figure 3

Histological findings of the skin lesion. Subcorneal bulla with mild acantholysis. No interface dermatitis was seen.

Figure 4

Histological findings of the oral lesion. Severe lichenoid dermatitis with dyskeratotic cells.

Figure 5

Direct immunofluorescence of the perilesional skin. Intercellular deposits of IgG throughout epidermis.

Figure 6

Direct immunofluorescence of the oral mucosa. Cell surface deposits of IgG in the lower layer of epithelium with ovoid bodies.

Figure 7

Indirect immunofluorescence was positive using rat bladder epithelium as a substrate.

Figure 8

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.

Figure 9

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).