scholarly journals Epidermal Transglutaminase (TGase 3) Is the Autoantigen of Dermatitis Herpetiformis

2002 ◽  
Vol 195 (6) ◽  
pp. 747-757 ◽  
Author(s):  
Miklós Sárdy ◽  
Sarolta Kárpáti ◽  
Barbara Merkl ◽  
Mats Paulsson ◽  
Neil Smyth
Keyword(s):  
Dermatitis Herpetiformis ◽  
Tissue Transglutaminase ◽  
Papillary Dermis ◽  
Gluten Sensitivity ◽  
Skin Symptoms ◽  
Related Enzyme ◽  
Small Intestinal ◽  
Type 3 ◽  
Epidermal Transglutaminase ◽  
Antibody Population

Gluten sensitivity typically presents as celiac disease, a common chronic small intestinal disorder. However, in certain individuals it is associated with dermatitis herpetiformis, a blistering skin disease characterized by granular IgA deposits in the papillary dermis. While tissue transglutaminase has been implicated as the major autoantigen of gluten sensitive disease, there has been no explanation as to why this condition appears in two distinct forms. Here we show that while sera from patients with either form of gluten sensitive disease react both with tissue transglutaminase and the related enzyme epidermal (type 3) transglutaminase, antibodies in patients having dermatitis herpetiformis show a markedly higher avidity for epidermal transglutaminase. Further, these patients have an antibody population specific for this enzyme. We also show that the IgA precipitates in the papillary dermis of patients with dermatitis herpetiformis, the defining signs of the disease, contain epidermal transglutaminase, but not tissue transglutaminase or keratinocyte transglutaminase. These findings demonstrate that epidermal transglutaminase, rather than tissue transglutaminase, is the dominant autoantigen in dermatitis herpetiformis and explain why skin symptoms appear in a proportion of patients having gluten sensitive disease.

scholarly journals Recent Advances in Dermatitis Herpetiformis

2012 ◽  
Vol 2012 ◽  
pp. 1-4 ◽  
Author(s):  
Kimiko Nakajima
Keyword(s):  
Celiac Disease ◽  
Small Bowel ◽  
Immune Responses ◽  
Dermatitis Herpetiformis ◽  
Gluten Sensitivity ◽  
Bullous Disease ◽  
Autoimmune Bullous Disease ◽  
Multifactorial Disorders ◽  
Epidermal Transglutaminase ◽  
Transglutaminase 3

Dermatitis herpetiformis is an autoimmune bullous disease that is associated with gluten sensitivity which typically presents as celiac disease. As both conditions are multifactorial disorders, it is not clear how specific pathogenetic mechanisms may lead to the dysregulation of immune responses in the skin and small bowel, respectively. Recent studies have demonstrated that IgA and antibodies against epidermal transglutaminase 3 play an important role in the pathogenesis of dermatitis herpetiformis. Here, we review recent immunopathological progress in understanding the pathogenesis of dermatitis herpetiformis.

scholarly journals Dermatitis Herpetiformis: An Update on Diagnosis, Disease Monitoring, and Management

Medicina ◽  
2021 ◽  
Vol 57 (8) ◽  
pp. 843
Author(s):  
Christopher N. Nguyen ◽  
Soo-Jung Kim
Keyword(s):  
Dermatitis Herpetiformis ◽  
Tissue Transglutaminase ◽  
The United States ◽  
Diagnostic Methods ◽  
Direct Immunofluorescence ◽  
Disease Monitoring ◽  
Gluten Free ◽  
Newly Diagnosed ◽  
Management Options ◽  
Epidermal Transglutaminase

Dermatitis herpetiformis (DH), Duhring disease, is caused by gluten sensitivity and affects 11.2 to 75.3 per 100,000 people in the United States and Europe with an incidence of 0.4 to 3.5 per 100,000 people per year. DH is characterized by a symmetrical blistering rash on the extensor surfaces with severe pruritus. The diagnosis continues to be made primarily by pathognomonic findings on histopathology, especially direct immunofluorescence (DIF). Recently, anti-epidermal transglutaminase (TG3) antibodies have shown to be a primary diagnostic serology, while anti-tissue transglutaminase (TG2) and other autoantibodies may be used to support the diagnosis and for disease monitoring. Newly diagnosed patients with DH should be screened and assessed for associated diseases and complications. A gluten-free diet (GFD) and dapsone are still mainstays of treatment, but other medications may be necessary for recalcitrant cases. Well-controlled DH patients, managed by a dermatologist, a gastroenterologist, and a dietician, have an excellent prognosis. Our review comprehensively details the current diagnostic methods, as well as methods used to monitor its disease course. We also describe both the traditional and novel management options reported in the literature.

scholarly journals Gluten and Functional Abdominal Pain Disorders in Children

Nutrients ◽  
2018 ◽  
Vol 10 (10) ◽  
pp. 1491 ◽  
Author(s):  
Alejandro Llanos-Chea ◽  
Alessio Fasano
Keyword(s):  
Abdominal Pain ◽  
Pediatric Patients ◽  
Functional Gastrointestinal Disorders ◽  
Gastrointestinal Symptoms ◽  
Gluten Sensitivity ◽  
Gluten Free ◽  
Functional Abdominal Pain ◽  
Adults And Children ◽  
Small Intestinal ◽  
Irritable Bowel

In children, functional gastrointestinal disorders (FGIDs) are common at all ages. Consumption of certain foods, particularly gluten, is frequently associated with the development and persistence of FGIDs and functional abdominal pain disorders (FAPDs) in adults and children. However, this association is not well defined. Even without a diagnosis of celiac disease (CD), some people avoid gluten or wheat in their diet since it has been shown to trigger mostly gastrointestinal symptoms in certain individuals, especially in children. The incidence of conditions such as non-celiac gluten sensitivity (NCGS) is increasing, particularly in children. On the other hand, CD is a chronic, autoimmune small intestinal enteropathy with symptoms that can sometimes be mimicked by FAPD. It is still unclear if pediatric patients with irritable bowel syndrome (IBS) are more likely to have CD. Abdominal, pain-associated FGID in children with CD does not seem to improve on a gluten-free diet. The threshold for gluten tolerance in patients with NCGS is unknown and varies among subjects. Thus, it is challenging to clearly distinguish between gluten exclusion and improvement of symptoms related solely to functional disorders.

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