Systemic Lupus Erythematosus Occurring in a Patient With Epidermolysis Bullosa Acquisita

Dotson, A. Dearl; Raimer, S.; Pursley, Terry V.; Tschen, Jaime A.

doi:10.1001/archderm.1981.01650070050025

Cited by 40 publications

(3 citation statements)

References 17 publications

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“…Another is the apparent association between SLE and EBA. Patients i have been described with concurrent SLE and EBA, SLE followed' by EBA, and EBA followed by SLE [41][42][43]. Of over 60 patients we have seen with autoimmunity to type VII collagen, approximately 30% have, have had, or develop SLE, which can hardly be viewed as a coincidental association given the relative infrequency of the conditions.…”

Section: Elationship Between Sle and H Autoimmunity To Type VII Collamentioning

confidence: 99%

Bullous SLE: A Phenotypically Distinctive but Immunologically Heterogeneous Bullous Disorder.

Gammon¹,

Briggaman²

1993

J Invest Dermatol

108

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Bullous systemic lupus erythematosus (SLE) is a rare blistering disease with a distinctive combination of clinical, histologic and immunopathologic features that together constitute a unique bullous disease phenotype. There appear to be at least two immunologically distinct subtypes of bullous SLE characterized by the presence or absence of circulating and/or tissue-bound basement membrane zone autoantibodies that recognize type VII collagen. The two subtypes are not clearly distinguishable except by indirect immunofluorescence and/or direct immunoelectron microscopy. In patients without circulating antibodies, immunoelectron microscopy is required to distinguish between the two subtypes. Patients with autoantibodies to type VII collagen are similar but not identical to patients with epidermolysis bullosa acquisita--another bullous disease associated with autoantibodies to type VII collagen. Autoantibodies to type VII collagen in patients with bullous SLE is only one of several lines of evidence that indicate autoimmunity to that protein and susceptibility to SLE are associated phenomena. In addition, there is emerging evidence for an association between epidermolysis bullous acquisita and SLE. There is also evidence that autoantibodies to type VII collagen are pathogenic in bullous SLE (and epidermolysis bullosa acquisita) and that their production is regulated by the class II major histocompatibility complex DR beta 1 allele, 1501 and possibly other DR beta 1 alleles that share a similar sequence of amino acids in the second hyper-variable region.

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Section: Elationship Between Sle and H Autoimmunity To Type VII Collamentioning

confidence: 99%

Bullous SLE: A Phenotypically Distinctive but Immunologically Heterogeneous Bullous Disorder.

Gammon¹,

Briggaman²

1993

J Invest Dermatol

108

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“…Some authors claimed that autoantibodies to type VII collagen are seen in BSLE patients whereas some assumed there is a particular association between EBA and SLE. Also, there have been reported cases with concurrent SLE and EBA, EBA followed by SLE, and SLE followed by EBA [35][36][37][38][39]. Some patients with SLE were reported that had autoantibodies to type VII collagen documented by indirect immunofluorescence (IIF) on split skin, immunoblotting, and immunoelectron microscopy (IEM) methods but no blistering disease [30].…”

Section: Immunopathogenesismentioning

confidence: 99%

Bullous Systemic Lupus Erythematosus and Cicatricial Pemphigoid

Ayvaz¹,

Gönül²,

Atay³

2018

Autoimmune Bullous Diseases

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Bullous systemic lupus erythematosus is a rare distinctive subepidermal bullous disease seen in patients with systemic lupus erythematosus (SLE). It has characteristical clinic, pathologic, and immunologic findings including antibodies to type VII collagen, laminin 332, laminin 331, and bullous pemphigoid antigen 230. Clinical presentation combined with histopathology, immunological testing, and concomitant diagnosis of SLE according to the criteria of American College of Rheumatology, are required to distinguish bullous SLE from these bullous diseases. In patients with bullous SLE, SLE disease progression and complications may be worse. Cicatricial pemphigoid is a chronic subepidermal blistering disease which is characterized by erosive lesions of mucous membranes and skin. Pathogenesis of cicatricial pemphigoid is characterized by linear deposition of Immunoglobulin G, A, or complement 3 along the epithelial basement membrane zone. The main target antigens are bullous pemphigoid antigens 180-230, laminin 331-332, type VII collagen, and β-4 integrin subunit. Cicatricial pemphigoid may lead to serious complications such as blindness and airway obstruction. Herein, clinical, histological, immunopathological features, the diagnosis and treatment of bullous SLE and cicatricial pemphigoid diseases are mentioned to raise awareness among the dermatologists about this important but rare heterogeneous bullous disease.

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“…EBA is a rare disease with an estimated incidence of 0.19–0.26 new cases/million inhabitants/year [6–9]. Autoantibodies against type VII collagen are also responsible for the skin blistering in a subgroup of patients with systemic lupus erythematosus [10–19]. Occasionally, EBA is associated with further autoimmune diseases, including rheumatoid arthritis and diabetes mellitus [20], as well as cryoglobulinemia [21] and psoriasis [22–24].…”

Section: Introductionmentioning

confidence: 99%

Autoimmunity against type VII collagen in inflammatory bowel disease

Hundorfean

Neurath

Sitaru

2009

J Cellular Molecular Medi

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Autoimmunity against type VII collagen, an adhesion molecule of the extracellular matrix in epithelial basement membranes, is causing the rare organ-specific epidermolysis bullosa acquisita (EBA). An intriguing association between EBA and inflammatory bowel disease (IBD) has been extensively documented over the last decades, but, because of the very low incidence of EBA, received little attention from physicians involved in the care of patients with IBD. More recently, autoantibodies against type VII collagen have been detected in up to 68% of IBD patients. Although these findings suggest that chronic intestinal inflammation in IBD predisposes for autoimmunity against type VII collagen, their relevance for the pathogenesis of both IBD and EBA is still unclear. In this review article, the main features of the association between IBD and EBA are presented and pathomechanistic hypotheses as well as future lines of investigation in this area are discussed. Future research should provide new pathomechanistic insights and will likely facilitate the development of more specific and effective immunotherapeutic strategies for both conditions.

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Systemic Lupus Erythematosus Occurring in a Patient With Epidermolysis Bullosa Acquisita

Cited by 40 publications

References 17 publications

Bullous SLE: A Phenotypically Distinctive but Immunologically Heterogeneous Bullous Disorder.

Bullous SLE: A Phenotypically Distinctive but Immunologically Heterogeneous Bullous Disorder.

Bullous Systemic Lupus Erythematosus and Cicatricial Pemphigoid

Autoimmunity against type VII collagen in inflammatory bowel disease

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