The use of skin substrates deficient in basement membrane (BM) molecules for the diagnosis of subepidermal autoimmune bullous diseases

Vodegel, RM; Kiss, Mária; Jong, Mcjm de; Pas, HH; Altmayer, Anita; Molnár, Katalin; Husz, S.; Meer, JB van der; Yancey, KB; Jonkman, M. F.

doi:10.1016/s0923-1811(98)83248-9

Cited by 23 publications

(28 citation statements)

References 0 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Of the 15 SSS‐positive patients, 12 (80%) had a positive ELISA index (Table ). To ascertain that the three with a negative coll VII ELISA (cases 2, 7, 12) did recognize coll VII we performed immunofluorescence knock‐out analysis . Two sera (cases 7 and 12) did not bind to coll VII‐deficient skin, but did bind to laminin‐332‐deficient skin confirming the presence of anti‐coll VII antibodies.…”

Section: Resultsmentioning

confidence: 99%

Low sensitivity of type VII collagen enzyme-linked immunosorbent assay in epidermolysis bullosa acquisita: serration pattern analysis on skin biopsy is required for diagnosis

et al. 2013

Self Cite

View full text Add to dashboard Cite

show abstract

Section: Resultsmentioning

confidence: 99%

Low sensitivity of type VII collagen enzyme-linked immunosorbent assay in epidermolysis bullosa acquisita: serration pattern analysis on skin biopsy is required for diagnosis

et al. 2013

Self Cite

View full text Add to dashboard Cite

show abstract

“…We have found 3 patients with EBA during the past 40 years. This is caused by IgG antibodies against type VII collagen [30, 31]. …”

Section: Resultsmentioning

confidence: 99%

Clinical Relevance of Autoantibodies in Patients with Autoimmune Bullous Dermatosis

Mihályi

Kiss

Kemény

et al. 2012

Clinical and Developmental Immunology

View full text Add to dashboard Cite

The authors present their experience related to the diagnosis, treatment, and followup of 431 patients with bullous pemphigoid, 14 patients with juvenile bullous pemphigoid, and 273 patients with pemphigus. The detection of autoantibodies plays an outstanding role in the diagnosis and differential diagnosis. Paraneoplastic pemphigoid is suggested to be a distinct entity from the group of bullous pemphigoid in view of the linear C3 deposits along the basement membrane of the perilesional skin and the “ladder” configuration of autoantibodies demonstrated by western blot analysis. It is proposed that IgA pemphigoid should be differentiated from the linear IgA dermatoses. Immunosuppressive therapy is recommended in which the maintenance dose of corticosteroid is administered every second day, thereby reducing the side effects of the corticosteroids. Following the detection of IgA antibodies (IgA pemphigoid, linear IgA bullous dermatosis, and IgA pemphigus), diamino diphenyl sulfone (dapsone) therapy is preferred alone or in combination. The clinical relevance of autoantibodies in patients with autoimmune bullous dermatosis is stressed.

show abstract

“…Skin substrates from Hallopeau-Siemens type of RDEB patients are used because the samples are deficient in C7. EBA sera have a lack of antibody labeling to the skin deficient in C7 along with a positive result on normal or BP-180 or laminin 5 deficient skins [67, 68]; however, this technique is limited by the availability of suitable skin samples from subjects with EB.…”

Section: Laboratory Testsmentioning

confidence: 99%

Epidermolysis bullosa acquisita

Gupta

Woodley

Chen

2012

Clinics in Dermatology

150

112

View full text Add to dashboard Cite

EBA is a rare, acquired, chronic subepidermal bullous disease of the skin and mucosa characterized by autoantibodies to type VII collagen structures, a major component of anchoring fibrils, that attach the epidermis onto the dermis. EBA patients have tissue-bound as well as circulating anti-type VII collagen autoantibodies that attack type VII collagen and result in a reduction or perturbation of normally functioning anchoring fibrils. Patients with EBA have skin fragility, blisters, erosions, scars, milia, and nail loss: all features reminiscent of genetic dystrophic epidermolysis bullosa. These anti-type VII collagen antibodies are “pathogenic” because when injected into mice, the mice develop an EBA-like blistering disease. In addition to the classical mechanobullous presentation, EBA also has several other distinct clinical syndromes similar to bullous pemphigoid, Brunsting-Perry pemphigoid, or cicatricial pemphigoid. Although treatment for EBA is often unsatisfactory, some therapeutic success has been achieved with colchicine, dapsone, plasmaphoresis, photopheresis, infliximab, and intravenous immunoglobulin.

show abstract

The use of skin substrates deficient in basement membrane (BM) molecules for the diagnosis of subepidermal autoimmune bullous diseases

Cited by 23 publications

References 0 publications

Low sensitivity of type VII collagen enzyme-linked immunosorbent assay in epidermolysis bullosa acquisita: serration pattern analysis on skin biopsy is required for diagnosis

Low sensitivity of type VII collagen enzyme-linked immunosorbent assay in epidermolysis bullosa acquisita: serration pattern analysis on skin biopsy is required for diagnosis

Clinical Relevance of Autoantibodies in Patients with Autoimmune Bullous Dermatosis

Epidermolysis bullosa acquisita

Contact Info

Product

Resources

About