Kindler Syndrome

Joey E. Lai-Cheong; John A. McGrath

doi:10.1007/978-3-662-45698-9_43

Kindler Syndrome

Joey E. Lai-Cheong
, John A. McGrath

Department of Dermatology

Research output: Chapter in Book/Report/Conference proceeding › Chapter

2 Citations (Scopus)

Abstract

Epidermolysis bullosa acquisita (EBA) is a rare subepidermal autoimmune bullous disease (AIBD) characterized by immune deposits on anchoring fibrils (AFs) of cutaneous and mucosal basement membrane zones (BMZ). It is due to circulating autoantibodies (Ab) directed to type VII collagen (C7). Clinical manifestations include a classical form with skin fragility, blisters and scars on trauma-prone surfaces, and misleading forms mimicking bullous pemphigoid (BP), mucous membrane pemphigoid (MMP), or linear immunoglobulin (Ig) A disease (LAD). Prognosis is related to extent of cutaneous lesions and ocular, laryngeal, and/or esophageal involvements. A frequent association between EBA and Crohn's disease has been observed. Specialized tests available in only certain laboratories are necessary to confirm its diagnosis such as immunoelectron microscopy (IEM), immunoblotting, or ELISA using recombinant proteins. This orphan disease is often misdiagnosed, and awareness programs for physicians and patients are mandatory.

Original language	English
Title of host publication	Blistering Diseases
Subtitle of host publication	Clinical Features, Pathogenesis, Treatment
Publisher	Springer Berlin Heidelberg
Pages	433-439
Number of pages	7
ISBN (Electronic)	9783662456989
ISBN (Print)	9783662456972
DOIs	https://doi.org/10.1007/978-3-662-45698-9_43
Publication status	Published - 2015 Jan 1

All Science Journal Classification (ASJC) codes

General Medicine

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10.1007/978-3-662-45698-9_43

Cite this

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title = "Kindler Syndrome",

abstract = "Epidermolysis bullosa acquisita (EBA) is a rare subepidermal autoimmune bullous disease (AIBD) characterized by immune deposits on anchoring fibrils (AFs) of cutaneous and mucosal basement membrane zones (BMZ). It is due to circulating autoantibodies (Ab) directed to type VII collagen (C7). Clinical manifestations include a classical form with skin fragility, blisters and scars on trauma-prone surfaces, and misleading forms mimicking bullous pemphigoid (BP), mucous membrane pemphigoid (MMP), or linear immunoglobulin (Ig) A disease (LAD). Prognosis is related to extent of cutaneous lesions and ocular, laryngeal, and/or esophageal involvements. A frequent association between EBA and Crohn's disease has been observed. Specialized tests available in only certain laboratories are necessary to confirm its diagnosis such as immunoelectron microscopy (IEM), immunoblotting, or ELISA using recombinant proteins. This orphan disease is often misdiagnosed, and awareness programs for physicians and patients are mandatory.",

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AU - McGrath, John A.

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N2 - Epidermolysis bullosa acquisita (EBA) is a rare subepidermal autoimmune bullous disease (AIBD) characterized by immune deposits on anchoring fibrils (AFs) of cutaneous and mucosal basement membrane zones (BMZ). It is due to circulating autoantibodies (Ab) directed to type VII collagen (C7). Clinical manifestations include a classical form with skin fragility, blisters and scars on trauma-prone surfaces, and misleading forms mimicking bullous pemphigoid (BP), mucous membrane pemphigoid (MMP), or linear immunoglobulin (Ig) A disease (LAD). Prognosis is related to extent of cutaneous lesions and ocular, laryngeal, and/or esophageal involvements. A frequent association between EBA and Crohn's disease has been observed. Specialized tests available in only certain laboratories are necessary to confirm its diagnosis such as immunoelectron microscopy (IEM), immunoblotting, or ELISA using recombinant proteins. This orphan disease is often misdiagnosed, and awareness programs for physicians and patients are mandatory.

AB - Epidermolysis bullosa acquisita (EBA) is a rare subepidermal autoimmune bullous disease (AIBD) characterized by immune deposits on anchoring fibrils (AFs) of cutaneous and mucosal basement membrane zones (BMZ). It is due to circulating autoantibodies (Ab) directed to type VII collagen (C7). Clinical manifestations include a classical form with skin fragility, blisters and scars on trauma-prone surfaces, and misleading forms mimicking bullous pemphigoid (BP), mucous membrane pemphigoid (MMP), or linear immunoglobulin (Ig) A disease (LAD). Prognosis is related to extent of cutaneous lesions and ocular, laryngeal, and/or esophageal involvements. A frequent association between EBA and Crohn's disease has been observed. Specialized tests available in only certain laboratories are necessary to confirm its diagnosis such as immunoelectron microscopy (IEM), immunoblotting, or ELISA using recombinant proteins. This orphan disease is often misdiagnosed, and awareness programs for physicians and patients are mandatory.

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SN - 9783662456972

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BT - Blistering Diseases

PB - Springer Berlin Heidelberg

ER -

Kindler Syndrome

Abstract

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