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

Niimi Y, Ohyama B, Di Zenzo G, Calabresi V, Hashimoto T, Kawana S - Dermatol Res Pract (2010)

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology, Nippon Medical School, 1-1-5, Sendagi, Bunkyo-ku, Tokyo 113-8603, Japan.

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.

No MeSH data available.


Related in: MedlinePlus

Histological findings of the skin lesion. Subcorneal bulla with mild acantholysis.  No interface dermatitis was seen.
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fig3: Histological findings of the skin lesion. Subcorneal bulla with mild acantholysis. No interface dermatitis was seen.

Mentions: A 49-year-old man presented with a 4-month history of oral lesion and a one-month history of skin lesions on the trunk. He had been admitted to the Department of Internal Medicine for evaluation of an abdominal mass with pain. His past history was unremarkable. On examination, he had blisters and erosions on the buccal mucosa, tongue and lips (Figure 1). The oral lesions did not extend onto the vermillion of the lips. He also had several scattered crusted and erosive lesions with small flaccid bullae on the chest and back (Figure 2). No lichenoid lesions were observed on skin. Biopsy of the flaccid bulla on the chest showed subcorneal bulla formation with mild acantholysis (Figure 3). No interface dermatitis was seen histologically. Histopathological findings of the oral mucosa revealed severe lichenoid dermatitis with dyskeratotic cells (Figure 4). 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 showed cell surface deposits of IgG and C3 in the lower layer of epithelium with ovoid bodies of 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) [1]. Anti-Dsg1 antibody index was 46 (positive) and anti-Dsg3 antibody index was 8 (negative) by ELISA using baculovirus protein. Anti-BP230 antibodies and anti-BP180 antibodies were negative by ELISA. By immunoblot analysis using normal human epidermal extract [3], the patient's serum reacted with BP230 and the 190 kDa periplakin, but not with the 210 kDa envoplakin (Figure 8). In contrast, by immunoprecipitation using radiolabeled cultured keratinocytes [1], 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).


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

Niimi Y, Ohyama B, Di Zenzo G, Calabresi V, Hashimoto T, Kawana S - Dermatol Res Pract (2010)

Histological findings of the skin lesion. Subcorneal bulla with mild acantholysis.  No interface dermatitis was seen.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC2913839&req=5

fig3: Histological findings of the skin lesion. Subcorneal bulla with mild acantholysis. No interface dermatitis was seen.
Mentions: A 49-year-old man presented with a 4-month history of oral lesion and a one-month history of skin lesions on the trunk. He had been admitted to the Department of Internal Medicine for evaluation of an abdominal mass with pain. His past history was unremarkable. On examination, he had blisters and erosions on the buccal mucosa, tongue and lips (Figure 1). The oral lesions did not extend onto the vermillion of the lips. He also had several scattered crusted and erosive lesions with small flaccid bullae on the chest and back (Figure 2). No lichenoid lesions were observed on skin. Biopsy of the flaccid bulla on the chest showed subcorneal bulla formation with mild acantholysis (Figure 3). No interface dermatitis was seen histologically. Histopathological findings of the oral mucosa revealed severe lichenoid dermatitis with dyskeratotic cells (Figure 4). 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 showed cell surface deposits of IgG and C3 in the lower layer of epithelium with ovoid bodies of 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) [1]. Anti-Dsg1 antibody index was 46 (positive) and anti-Dsg3 antibody index was 8 (negative) by ELISA using baculovirus protein. Anti-BP230 antibodies and anti-BP180 antibodies were negative by ELISA. By immunoblot analysis using normal human epidermal extract [3], the patient's serum reacted with BP230 and the 190 kDa periplakin, but not with the 210 kDa envoplakin (Figure 8). In contrast, by immunoprecipitation using radiolabeled cultured keratinocytes [1], 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).

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology, Nippon Medical School, 1-1-5, Sendagi, Bunkyo-ku, Tokyo 113-8603, Japan.

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.

No MeSH data available.


Related in: MedlinePlus