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Case Report/Case Series |

Two Cases of Pemphigus Vegetans With IgG Anti–Desmocollin 3 Antibodies

Hiroshi Saruta, MD1; Norito Ishii, MD1; Kwesi Teye, PhD1; Fumitake Ono, MD1; Bungo Ohyama, MD, PhD1; Hiroshi Koga, MD1; Chika Ohata, MD1; Minao Furumura, MD, PhD1; Daisuke Tsuruta, MD, PhD1; Takashi Hashimoto, MD1
[+] Author Affiliations
1Department of Dermatology, Kurume University School of Medicine, and Kurume University Institute of Cutaneous Cell Biology, Kurume, Fukuoka, Japan
JAMA Dermatol. 2013;149(10):1209-1213. doi:10.1001/jamadermatol.2013.5244.
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Importance  Pemphigus vegetans shows clinically vegetating and/or pustular skin lesions mainly on the intertriginous areas and histopathologically neutrophilic and eosinophilic pustules in the epidermis. Pemphigus vegetans shows IgG reactivity mainly with desmoglein (Dsg) 3, but also with other autoantigens, including Dsg1 and desmocollins (Dscs).

Observations  We examined antigen profiles in 2 cases of pemphigus vegetans. (1) A women in her 80s presented with typical vegetating skin lesions on the right inguinal region with typical histopathological features. Immunoblotting using normal human epidermal extracts detected IgG antibodies to Dsg1 and Dscs. Enzyme-linked immunosorbent assays (ELISAs) revealed IgG antibodies to Dsg1 but not to Dsg3. Complementary DNA (cDNA) transfection method to COS-7 cells and novel ELISAs using eukaryotic recombinant proteins of human Dsc1, Dsc2, and Dsc3 confirmed specific IgG reactivity with Dsc3. (2) A women in her 70s presented with pustular skin lesions on the left fingers with typical histopathological features. Immunoblotting and ELISAs did not detect antibodies to either Dsg1 or Dsg3. Conversely, immunoblotting detected IgG antibodies to Dscs, cDNA transfection method revealed IgG reactivity only with Dsc3, and findings from ELISAs showed that IgG reacted weakly with Dsc2 and strongly with Dsc3.

Conclusions and Relevance  Autoantibodies to Dscs, particularly to Dsc3, may play a pathogenic role in some cases of pemphigus vegetans.

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Figure 1.
Case 1

A, Clinical appearance. B and C, Histopathological features (hematoxylin-eosin, original magnification ×100 [B], ×400 [C]). D, Direct immunofluorescence for C3. E, Indirect immunofluorescence of monkey esophagus sections for IgG antibodies. F, The results of immunoblotting of normal human epidermal extracts for IgG antibodies. A control pemphigus vulgaris serum sample reacted with both the 160-kDa Dsg1 and 130-kDa Dsg3 (lane 1, PV control); a control paraneoplastic pemphigus serum sample reacted with both the 210-kDa envoplakin and the 190-kDa periplakin (lane 2, PNP control); a control bullous pemphigoid serum sample reacted with both the 230-kDa BP230 and the 180-kDa BP180 (lane 3, BP control); anti-desmocollin monoclonal antibody 52-3B reacted with the 110-kDa a-form and the 100-kDa b-form of desmocollins (lane 4, Dsc); and IgG antibodies of case 1 reacted clearly with Dsg1 and Dscs (lane 5, Patient). G, Complementary DNA transfection method for IgG antibodies to Dsc3.

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Figure 2.
Case 2

A, Clinical appearance. B and C, Histopathological features (hematoxylin-eosin, original magnification, ×100 [B] and ×400 [C]). D, Indirect immunofluorescence of monkey esophagus sections for IgG antibodies. E, The results of immunoblotting of normal human epidermal extracts for IgG antibodies. The explanation for each lane is the same as that in Figure 1. F, Complementary DNA transfection method for IgG antibodies to Dsc3.

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