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Clinical and Experimental Dermatology Oct 2019Dermatitis herpetiformis (DH) is a cutaneous manifestation of coeliac disease (CD), which causes an itching and blistering rash, typically on the elbows, knees and... (Review)
Review
Dermatitis herpetiformis (DH) is a cutaneous manifestation of coeliac disease (CD), which causes an itching and blistering rash, typically on the elbows, knees and buttocks. DH and CD share a similar genetic background, small bowel mucosal alterations, and an autoimmune response against tissue transglutaminase in the serum and small bowel. DH is typically diagnosed during adulthood, and it is slightly more common among males than females. The incidence of DH seems to be decreasing, in contrast to the detected four-fold increase in the incidence of CD. In addition to typical clinical picture, diagnosis of DH relies on the demonstration by direct immunofluorescence of pathognomonic granular IgA deposits in the papillary dermis. Circulating tissue transglutaminase antibodies support the diagnosis, but their absence does not exclude DH. Obtainment of small bowel mucosal biopsies is not necessary when DH is diagnosed, but if performed, the majority of patients are found to have villous atrophy, and even those with normal villous architecture evince CD-type inflammation. The treatment of choice in DH is a strict, life-long adherence to a gluten-free diet (GFD). In addition to alleviating the symptoms of DH and healing the small bowel mucosal damage, a GFD increases the quality of life for patients, and decreases the risk for lymphoma in DH. Further, the mortality rate of patients with DH treated with a GFD seems to be lower than that of the general population. However, as changing to a GFD has a rather slow effect on the DH rash, patients with severe skin symptoms should additionally be treated with dapsone medication. This review article is based on a presentation given at the British Society for Medical Dermatology blistering skin diseases meeting 2019.
Topics: Celiac Disease; Dermatitis Herpetiformis; Diet, Gluten-Free; Humans; Prognosis
PubMed: 31093998
DOI: 10.1111/ced.13992 -
BMJ (Clinical Research Ed.) Apr 2014
Review
Topics: Dermatitis Herpetiformis; Diet, Gluten-Free; Humans; Skin
PubMed: 24740905
DOI: 10.1136/bmj.g2557 -
Medicina (Kaunas, Lithuania) Aug 2021Dermatitis 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... (Review)
Review
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.
Topics: Autoantibodies; Celiac Disease; Dermatitis Herpetiformis; Diet, Gluten-Free; Humans; Immunoglobulin A; Prognosis
PubMed: 34441049
DOI: 10.3390/medicina57080843 -
Skin Therapy Letter 2013Dermatitis herpetiformis (DH) is an autoimmune, pleiomorphic, papulovesicular disorder associated with celiac disease and gluten sensitivity. DH is characterized by... (Review)
Review
Dermatitis herpetiformis (DH) is an autoimmune, pleiomorphic, papulovesicular disorder associated with celiac disease and gluten sensitivity. DH is characterized by subepidermal bullae on hematoxylin and eosin staining and granular immunoglobulin A deposits in the dermal papillae using the direct immunofluorescence method. Antibodies to tissue transglutaminase and epidermal transgulatminase can be measured serologically, although biopsy is still required for definitive diagnosis of DH. Gluten free diet (GFD) is the first-line therapeutic approach that can alleviate both cutaneous and intestinal manifestations of this condition, while dapsone and sulfones target the skin eruption only. Combined therapy with GFD and dapsone is an initial treatment of choice to control the cutaneous manifestations of DH. This article will provide a comprehensive review of DH, including its epidemiology, clinical and pathological findings, diagnostic evaluation, and management.
Topics: Biopsy; Combined Modality Therapy; Dapsone; Dermatitis Herpetiformis; Diet, Gluten-Free; Fluorescent Antibody Technique, Direct; Humans; Leprostatic Agents; Sulfones; Transglutaminases
PubMed: 23674144
DOI: No ID Found -
Der Hautarzt; Zeitschrift Fur... Apr 2019Dermatitis herpetiformis (DH) is a genetically determined, gluten sensitive autoimmune bullous dermatosis related to celiac disease in which granular, insoluble... (Review)
Review
Dermatitis herpetiformis (DH) is a genetically determined, gluten sensitive autoimmune bullous dermatosis related to celiac disease in which granular, insoluble aggregates in the papillary dermis of epidermal transglutaminase (TG3), immunoglobulin A (IgA), and fibrinogen are present. Detection of the dermal IgA-TG3 immune complex is the gold standard of diagnosis. DH develops in a subpopulation of patients with gluten sensitive enteropathy, characterized by itching, erythematous, excoriated papules showing characteristic distribution over the knees, elbows and buttocks; vesicles are rarely seen. The primary therapy of DH is a strict, lifelong gluten-free diet, and it may be necessary to temporarily give dapsone in case of severe symptoms.
Topics: Celiac Disease; Dermatitis Herpetiformis; Diet, Gluten-Free; Glutens; Humans; Transglutaminases
PubMed: 30868254
DOI: 10.1007/s00105-019-4378-8 -
Revista Medica de Chile Sep 2021Dermatitis herpetiformis is an autoimmune chronic blistering disease, considered a skin manifestation of celiac disease. Being both conditions multifactorial, they share... (Review)
Review
Dermatitis herpetiformis is an autoimmune chronic blistering disease, considered a skin manifestation of celiac disease. Being both conditions multifactorial, they share some genetic traits and pathogenic mechanisms, which are responsible for the typical skin and gastrointestinal manifestations. In dermatitis herpetiformis, skin and other lesions heal after gluten-free diet and reappear shortly after its reintroduction to complete diet. Prevalence of celiac disease is 1% in the population, and approximately 13% of patients with the disease develop dermatitis herpetiformis. Diagnosis of celiac disease has progressively increased in recent decades, while clinical manifestations become more and more diverse. Given the current high frequency of skin lesions in celiac patients, in this review we update relevant aspects of the epidemiology, pathogenesis, clinical presentations, treatment and follow up of dermatitis herpetiformis, as a contribution to improve the management of both conditions.
Topics: Celiac Disease; Dermatitis Herpetiformis; Humans; Skin
PubMed: 35319687
DOI: 10.4067/S0034-98872021000901330 -
International Journal of Dermatology Aug 2003
Review
Topics: Dermatitis Herpetiformis; Humans; Intestines
PubMed: 12890100
DOI: 10.1046/j.1365-4362.2003.01804.x -
Anais Brasileiros de Dermatologia 2013Dermatitis herpetiformis (DH) or Duhring-Brocq disease is a chronic bullous disease characterized by intense itching and burning sensation in the erythematous papules... (Review)
Review
Dermatitis herpetiformis (DH) or Duhring-Brocq disease is a chronic bullous disease characterized by intense itching and burning sensation in the erythematous papules and urticarial plaques, grouped vesicles with centrifuge growth, and tense blisters. There is an association with the genotypes HLA DR3, HLA DQw2, found in 80-90% of cases. It is an IgA-mediated cutaneous disease, with immunoglobulin A deposits appearing in a granular pattern at the top of the dermal papilla in the sublamina densa area of the basement membrane, which is present both in affected skin and healthy skin. The same protein IgA1 with J chain is found in the small intestinal mucosa in patients with adult celiac disease, suggesting a strong association with DH. Specific antibodies such as antiendomysium, antireticulina, antigliadin and, recently identified, the epidermal and tissue transglutaminase subtypes, as well as increased zonulin production, are common to both conditions, along with gluten-sensitive enteropathy and DH. Autoimmune diseases present higher levels of prevalence, such as thyroid (5-11%), pernicious anemia (1-3%), type 1 diabetes (1-2%) and collagen tissue disease. The chosen treatment is dapsone and a gluten-free diet.
Topics: Celiac Disease; Dermatitis Herpetiformis; Humans; Immunoglobulin A; Skin
PubMed: 24068131
DOI: 10.1590/abd1806-4841.20131775 -
The Journal of Investigative Dermatology Dec 1992
Review
Topics: Dermatitis Herpetiformis; Dermatology; Humans
PubMed: 1469303
DOI: 10.1111/1523-1747.ep12614868 -
Clinics in Dermatology 2001