A subepidermal bullous eruption associated with IgG autoantibodies to a 200 kd dermal antigen: The first case report from the United States

Mascaró, José M.; Zillikens, Detlef; Giudice, George J.; Caux, F.; Fleming, Matthew G.; Katz, Henry M.; Díaz, Luis A.

doi:10.1016/s0190-9622(00)90101-4

Cited by 37 publications

(42 citation statements)

References 21 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…The oral cavity and genital mucosa are often affected. Histological examination shows subepidermal bullae with a predominant neutrophilic infiltration both in the blister fluid and along the BMZ, but papillary microabscess formation is not seen [9]. A relationship between type VII collagen and this p200 has been suggested.…”

Section: Discussionmentioning

confidence: 99%

A Case of Epidermolysis Bullosa Acquisita with Autoantibody to Anti-p200 Pemphigoid Antigen and Exfoliative Esophagitis

et al. 2006

View full text Add to dashboard Cite

Background: Some cases of a subepidermal blistering disease associated with autoantibodies to more than two antigens have been reported. Observation: A 52-year-old Japanese woman had pruritic blisters on almost the whole body as well as erosive lesions in the oral cavity and esophagus. A histological finding was subepidermal bullae. Direct immunofluorescence (IF) revealed a linear deposition of IgG, IgM and C3 at the epidermal basement membrane zone (BMZ). Indirect IF using human skin split by 1 M NaCl as a substrate showed IgG antibody reactive with the dermal side. By immunoblot analysis using normal human dermal extract, the 200-kDa and 290-kDa bands were detected. Indirect IF did not show any anti-BMZ antibody activity, when using the skin of the patient with recessive dystrophic epidermolysis bullosa as a substrate. Conclusion: We regarded our case as epidermolysis bullosa acquisita with autoantibody to anti-p200 pemphigoid antigen. This is the second case in the literature associated with autoantibodies to these two antigens.

show abstract

Section: Discussionmentioning

confidence: 99%

A Case of Epidermolysis Bullosa Acquisita with Autoantibody to Anti-p200 Pemphigoid Antigen and Exfoliative Esophagitis

et al. 2006

View full text Add to dashboard Cite

show abstract

“…Four of these patients have been described in detail elsewhere (Zillikens et al, 1996Mascaro et al, 2000;Egan et al, 2002). All patients ful¢lled the following criteria: (1) linear deposits of IgG and C3 along the DEJ as detected by direct immuno£uorescence of perilesional skin biopsies; (2) linear deposition of IgG along the dermal side of NaCl-split normal human skin by indirect immuno£uorescence microscopy; and (3) circulating antibodies against a 200-kDa protein by immunoblotting of extracts of normal human dermis.…”

Section: Methodsmentioning

confidence: 99%

The Autoantigen of Anti-p200 Pemphigoid Is an Acidic Noncollagenous N-Linked Glycoprotein of the Cutaneous Basement Membrane

Shimanovich

Hirako

Sitaru

et al. 2003

Journal of Investigative Dermatology

Self Cite

View full text Add to dashboard Cite

Anti-p200 pemphigoid is an autoimmune subepidermal blistering disease characterized by autoantibodies to a 200-kDa protein (p200) of the dermal-epidermal junction (DEJ). p200 has been demonstrated to be distinct from all major DEJ autoantigens and is thought to be important for cell-matrix adhesion. This study provides the first biochemical characterization of p200. Differential extraction experiments demonstrated that efficient recovery of p200 from the dermis was strongly dependent on the presence of reducing agents, suggesting that it forms highly insoluble oligomers and/or is extensively cross-linked to other extracellular matrix components by disulfide bonding. p200 was resistant to digestion with bacterial collagenase, whereas this treatment did degrade major collagenous proteins of the dermis, including type I, VI, and VII collagen. This finding firmly established the noncollagenous nature of p200. N-Glycosidase F reduced the molecular size of the p200 autoantigen from 200 to 190 kDa without decreasing its immunoreactivity. In contrast, digestion of p200 with neuraminidase, O-glycosidase, chondroitinase ABC, and heparitinase I had no effect on its electrophoretic mobility. These data suggest that the p200 molecule contains N-glycans but lacks O-linked oligosaccharides and chondroitin/heparan sulfate side chains. Two-dimensional gel electrophoresis demonstrated that p200 is an acidic protein with an isoelectric point of 5.4 to 5.6. Six different p200-specific sera recognized an identical protein spot of two-dimensionally separated dermal extracts, confirming that patients with this novel autoimmune disease indeed form a single pathobiochemical entity.

show abstract

“…Anti-p200 pemphigoid is a recently described autoimmune bullous disease with autoantibodies against a 200-kD dermal antigen [1, 2, 3, 4, 5, 6, 7, 8, 9, 10]. Histologically, these cases show subepidermal blisters with neutrophil and eosinophil infiltration, and by indirect immunofluorescence (IF) there are circulating IgG autoantibodies against the dermal side of skin split with NaCl.…”

Section: Introductionmentioning

confidence: 99%

A Case of Anti-p200 Pemphigoid with Autoantibodies against both a Novel 200-kD Dermal Antigen and the 290-kD Epidermolysis bullosa acquisita Antigen

et al. 2004

View full text Add to dashboard Cite

Background: Anti-p200 pemphigoid with autoantibodies against the 200-kD dermal antigen has recently been identified. Objectives: Our patient showed small and tense blisters on her face and trunk. Methods: Immunoblotting (IB), using extracts of normal human epidermis and dermal skin, and immunoelectron microscopy (IEM), using normal human skin, were performed using the patient’s serum. Results: IB analysis showed that the patient’s serum did not react with 180-kD bullous pemphigoid (BP180) or BP230 antigens; however, IgG autoantibodies in the patient’s serum reacted with a 200-kD dermal antigen as well as the 290-kD epidermolysis bullosa acquisita (EBA) antigen. IEM showed that IgG antibodies in the patient’s serum bound to the lamina lucida, as well as both the lamina densa and sublamina densa. After the treatment with prednisolone, the 290-kD protein reactivity decreased rapidly; however, the 200-kD protein band was still observed. Conclusion: We describe a rare case with immunoreactive autoantibodies against both a novel dermal 200-kD autoantigen and the 290-kD EBA antigen.

show abstract

A subepidermal bullous eruption associated with IgG autoantibodies to a 200 kd dermal antigen: The first case report from the United States

Cited by 37 publications

References 21 publications

A Case of Epidermolysis Bullosa Acquisita with Autoantibody to Anti-p200 Pemphigoid Antigen and Exfoliative Esophagitis

A Case of Epidermolysis Bullosa Acquisita with Autoantibody to Anti-p200 Pemphigoid Antigen and Exfoliative Esophagitis

The Autoantigen of Anti-p200 Pemphigoid Is an Acidic Noncollagenous N-Linked Glycoprotein of the Cutaneous Basement Membrane

A Case of Anti-p200 Pemphigoid with Autoantibodies against both a Novel 200-kD Dermal Antigen and the 290-kD Epidermolysis bullosa acquisita Antigen

Contact Info

Product

Resources

About