Tags

Type your tag names separated by a space and hit enter

A novel variant of acquired epidermolysis bullosa with autoantibodies against the central triple-helical domain of type VII collagen.
Lab Invest. 1997 Dec; 77(6):623-32.LI

Abstract

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.

Authors+Show Affiliations

Department of Dermatology, Asahikawa Medical College, Japan.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

9426400

Citation

Tanaka, H, et al. "A Novel Variant of Acquired Epidermolysis Bullosa With Autoantibodies Against the Central Triple-helical Domain of Type VII Collagen." Laboratory Investigation; a Journal of Technical Methods and Pathology, vol. 77, no. 6, 1997, pp. 623-32.
Tanaka H, Ishida-Yamamoto A, Hashimoto T, et al. A novel variant of acquired epidermolysis bullosa with autoantibodies against the central triple-helical domain of type VII collagen. Lab Invest. 1997;77(6):623-32.
Tanaka, H., Ishida-Yamamoto, A., Hashimoto, T., Hiramoto, K., Harada, T., Kawachi, Y., Shimizu, H., Tanaka, T., Kishiyama, K., Höpfner, B., Takahashi, H., Iizuka, H., & Bruckner-Tuderman, L. (1997). A novel variant of acquired epidermolysis bullosa with autoantibodies against the central triple-helical domain of type VII collagen. Laboratory Investigation; a Journal of Technical Methods and Pathology, 77(6), 623-32.
Tanaka H, et al. A Novel Variant of Acquired Epidermolysis Bullosa With Autoantibodies Against the Central Triple-helical Domain of Type VII Collagen. Lab Invest. 1997;77(6):623-32. PubMed PMID: 9426400.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - A novel variant of acquired epidermolysis bullosa with autoantibodies against the central triple-helical domain of type VII collagen. AU - Tanaka,H, AU - Ishida-Yamamoto,A, AU - Hashimoto,T, AU - Hiramoto,K, AU - Harada,T, AU - Kawachi,Y, AU - Shimizu,H, AU - Tanaka,T, AU - Kishiyama,K, AU - Höpfner,B, AU - Takahashi,H, AU - Iizuka,H, AU - Bruckner-Tuderman,L, PY - 1998/1/14/pubmed PY - 1998/1/14/medline PY - 1998/1/14/entrez SP - 623 EP - 32 JF - Laboratory investigation; a journal of technical methods and pathology JO - Lab Invest VL - 77 IS - 6 N2 - 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. SN - 0023-6837 UR - https://www.unboundmedicine.com/medline/citation/9426400/A_novel_variant_of_acquired_epidermolysis_bullosa_with_autoantibodies_against_the_central_triple_helical_domain_of_type_VII_collagen_ L2 - http://ovidsp.ovid.com/ovidweb.cgi?T=JS&PAGE=linkout&SEARCH=9426400.ui DB - PRIME DP - Unbound Medicine ER -