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A
Novel Variant of Acquired Epidermolysis Bullosa with Autoantibodies against
the Central Triple-Helical Domain of Type VII Collagen |
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Hikaru Tanaka,
Akemi Ishida-Yamamoto, Takashi Hashimoto, Kazuyo Hiramoto, Takashi Harada,
Yasuhiro Kawachi, Hiroshi Shimizu, Toshihiro Tanaka, Kazunori Kishiyama,
Bianca HAdopfner, Hidetoshi Takahashi, Hajime Iizuka, and Leena Bruckner-Tuderman
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Department
of Dermatology (HTan, AI-Y, HTak, HI), Asahikawa Medical College, Asahikawa;
Kurume University School of Medicine (THas), Fukuoka; Tokyo Women's Medical
College Daini Hospital (KH, THar), Tokyo; Institute of Clinical Medicine
(YK), University of Tsukuba, Tsukuba; Keio University School of Medicine
(HS), Tokyo; Faculty of Medicine (TT), Kyoto University Medical School,
Kyoto; and Kitami Red Cross Hospital (KK), Kitami, Japan; and University
of MAdunster (BH, LBT), MAdunster, Germany |
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Epidermolysis bullosa acquisita
and bullous systemic lupus erythematosus are autoimmune bullous disorders,
with tissue-bound and circulating autoantibodies reactive with the noncollagenous
NC1 domain of type VII collagen (C-VII). Here, we describe a novel acquired
bullous dermatosis with autoantibodies against the triple-helical domain
of C-VII. Three patients, all Japanese children, presented with widespread
inflammatory tense blisters. Histologically, subepidermal tissue separation
was noted with inflammatory infiltrate in the superficial dermis. Direct
immunofluorescence staining revealed linear IgG/C3 deposits along the dermal-epidermal
junction. Circulating IgG anti-basement membrane zone autoantibodies stained
the dermal side of normal skin separated with 1 m NaCl. Direct and indirect
immunoelectron microscopy using colloidal gold labeling showed that patient
sera reacted with anchoring fibrils. The gold particles were localized both
near the lamina densa and on the central banded portion of the fibrils.
The sera reacted with C-VII in immunoblots. Epitope analyses with natural
and recombinant fragments of C-VII disclosed that the sera did not recognize
the NC1 domain of C-VII, but the central triple-helical domain of this anchoring
fibril protein. Thus, the present probands show a hitherto unrecognized
variant of epidermolysis bullosa acquisita, with autoantibodies against
epitopes in the collagenous domain of C-VII. |
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