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The treatment of choice for dermatitis herpetiformis (DH), a cutaneous manifestation of coeliac disease, is a life-long gluten-free diet (GFD). In a GFD, wheat, rye and barley should be strictly avoided, but the role of oats is more controversial. This study aimed to investigate the safety and long-term quality of life and health effects of oat consumption in 312 long-term treated DH patients. Baseline data were gathered from patient records and follow-up data from questionnaires or interviews, and validated questionnaires were used to assess quality of life. We found that altogether 256 patients (82%) were consuming oats as part of their GFD at the follow-up. Long-term follow-up data showed that there were no differences in the presence of long-term illnesses, coeliac disease complications or the usage of medication between those consuming and not consuming oats. However, oat consumers had a better quality of life and reported ongoing gastrointestinal symptoms less frequently (4% vs 19%, p = 0.004) at the follow-up than those not consuming oats. The study established that oats are safe for DH patients and in the long-term seem to improve the quality of life of DH patients.

Dermatitis herpetiformis (DH) is an extraintestinal manifestation of celiac disease causing an itchy, blistering rash. Granular IgA deposits in the skin are pathognomonic for DH, and the treatment of choice is a lifelong gluten-free diet (GFD). Preliminary evidence suggests that there are patients with DH who redevelop gluten tolerance after adherence to a GFD treatment. To evaluate this, we performed a 12-month gluten challenge with skin and small-bowel mucosal biopsy samples in 19 patients with DH who had adhered to a GFD for a mean of 23 years. Prechallenge biopsy was negative for skin IgA and transglutaminase 3 deposits in 16 patients (84%) and indicated normal villous height-to-crypt depth ratios in the small bowel mucosa in all 19 patients. The gluten challenge caused a relapse of the rash in 15 patients (79%) in a mean of 5.6 months; of these 15 patients, 13 had skin IgA and transglutaminase 3 deposits, and 12 had small-bowel villous atrophy. In addition, three patients without rash or immune deposits in the skin developed villous atrophy, whereas one patient persisted without any signs of relapse. In conclusion, 95% of the patients with DH were unable to tolerate gluten even after long-term adherence to a GFD. Therefore, lifelong GFD treatment remains justified in all patients with DH., (Copyright © 2019 The Authors. Published by Elsevier Inc. All rights reserved.)

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., (© 2019 British Association of Dermatologists.)

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.

Background: Indian data on dermatitis herpetiformis (DH) is not available. The aim of this study was to investigate the demographic and clinicopathological characteristics of patients with DH and to study its association with other autoimmune diseases., Methods: All data were collected from case record forms of patients registered in immunobullous disease clinic of our institute. The diagnosis was based on characteristic clinical and immuno/histopathological features., Results: A total of 65 patients were included, which constituted 9.47% of the registered patients in the immunobullous disease clinic over 3.5 years. The male to female ratio was 1.4 : 1; the average age was 44.35 ± 15.52 years. Direct immunofluorescence showed granular IgA deposits at the papillary tips in 83.07% and basement membrane zone in 12.3% patients. Sixteen (24.1%) patients had associated celiac disease, and 15 (23.07%) patients had other autoimmune comorbidities such as hypothyroidism. Forty percent of patients on strict gluten-free diet achieved remission in 2 years, while 35.4% had frequent relapses as they continued gluten intake., Conclusions: Dermatitis herpetiformis is not a rare disease in northern India as previously believed. The clinical, histological, and immunopathological characteristics of Indian DH patients are similar to those reported in Caucasian populations. The limitations of our study include an absence of genetic testing for HLA-DQ2 or DQ8, nonavailability of kits for detecting IgA specific for epidermal transglutaminase (IgA eTG), and short follow-up period., (© 2018 The International Society of Dermatology.)

Dermatitis herpetiformis (DH) is a common extraintestinal manifestation of coeliac disease presenting with itchy papules and vesicles on the elbows, knees, and buttocks. Overt gastrointestinal symptoms are rare. Diagnosis of DH is easily confirmed by immunofluorescence biopsy showing pathognomonic granular immunoglobulin A (IgA) deposits in the papillary dermis. A valid hypothesis for the immunopathogenesis of DH is that it starts from latent or manifest coeliac disease in the gut and evolves into an immune complex deposition of high avidity IgA epidermal transglutaminase (TG3) antibodies, together with the TG3 enzyme, in the papillary dermis. The mean age at DH diagnosis has increased significantly in recent decades and presently is 40⁻50 years. The DH to coeliac disease prevalence ratio is 1:8 in Finland and the United Kingdom (U.K.). The annual DH incidence rate, currently 2.7 per 100,000 in Finland and 0.8 per 100,000 in the U.K., is decreasing, whereas the reverse is true for coeliac disease. The long-term prognosis of DH patients on a gluten-free diet is excellent, with the mortality rate being even lower than for the general population.

Dermatitis herpetiformis (DH) is an itchy blistering skin disease with predilection sites on elbows, knees, and buttocks. Diagnosis is confirmed by showing granular immunoglobulin A deposits in perilesional skin. DH is one manifestation of coeliac disease; the skin symptoms heal with gluten free diet (GFD) and relapse on gluten challenge. Of the first-degree relatives, 5% may be affected by either condition. Tissue transglutaminase (TG2) is the autoantigen in coeliac disease and epidermal transglutaminase (TG3) in DH. Both diseases conditions exhibit TG2-specific autoantibodies in serum and small bowel mucosa; patients with DH have IgA-TG3 in the skin. There are some divergencies between these two phenotypes. One-fourth of DH patients do not have small bowel mucosal villous atrophy, but virtually all have coeliac-type inflammatory changes. The skin symptoms respond slowly to GFD. The incidence of coeliac disease is increasing, whereas the opposite is true for DH. A female predominance is evident in coeliac disease, while DH may be more common in males. Coeliac disease carries the risk of small intestinal T-cell lymphoma; in DH B-cell lymphomas at any site may prevail. Adult coeliac disease carries a slightly increased elevated mortality risk, whereas in DH, the relative mortality rate is significantly decreased. Key messages Dermatitis herpetiformis is a cutaneous manifestation of coeliac disease; both conditions are genetically determined and gluten-dependent. Gastrointestinal symptoms and the degree of villous atrophy are less obvious in dermatitis herpetiformis than in coeliac disease. Both show tissue transglutaminase (TG2) specific autoantibodies in serum and small bowel mucosa. In addition, TG3-targeted IgA antibodies are found in the skin of DH patients Both conditions carry an increased elevated risk of lymphoma, in coeliac disease small intestinal T-cell lymphoma, in dermatitis herpetiformis mainly B-cell lymphoma at various sites. Coeliac disease is currently eight times more common that DH; the incidence of DH is decreasing in contrast to that of coeliac disease, where it is increasing.

Dermatitis herpetiformis (DH) is an extraintestinal manifestation of coeliac disease. The burden of illness in untreated coeliac disease is known to be considerable, but corresponding evidence for DH is lacking. In this study the burden of DH was evaluated prospectively in 52 patients newly diagnosed with DH using a study questionnaire and a validated Psychological General Well-Being (PGWB) questionnaire. The PGWB scores were compared with those of 110 healthy controls. Quality of life was significantly (p < 0.001) lower among patients with DH at the time of diagnosis, but after 1 year on a gluten-free diet their quality of life was at same level as that of the controls. The presence of gastrointestinal symptoms was shown to significantly increase the burden of untreated DH. We conclude that there is a significant burden related to untreated, but not to treated, DH, and the burden is even greater among DH patients with gastrointestinal symptoms.

The market of gluten-free bakery products is considerably growing since better diagnostic methods allow identifying an increasing number of people suffering coeliac disease and other gluten-related disorders such as dermatitis herpetiformis, gluten ataxia, wheat allergy and non-coeliac gluten sensitivity. The only and safe treatment available nowadays for these types of disorders is to follow a strict and permanent lifelong gluten-free diet. Beside the people needing to follow a gluten-free diet for health reasons, a new segment of consumers has arisen who consume gluten-free products as a lifestyle choice. Among the bakery products, bread is a major staple food consumed daily all over the world. The dough and bread quality characteristics (such as gas retaining ability, mixing tolerance, resistance to stretch and extensibility and crumb structure) are mostly attributed to the presence of gluten. Despite the improved quality of gluten-free breads in the last number of years, most products on the market are still described as low quality product. In addition to the low overall quality of gluten-free products, the nutritional value of a large number of them is quite poor. In this context, this review gives an overview on the consumers, which need to follow a gluten-free diet for health reasons. The trends in this gluten-free bakery segment will also be reviewed based on the current analysis of marketing studies. An overview of the major ingredients used in gluten-free bread products will be given. The choice of the ingredients discussed in this paper is based on a comprehensive study of the leading gluten-free breads available on the market, as well as a detailed study of the scientific literature. The impact of the various ingredients on bread-making process and bread quality is also part of this review. Major emphasis will be placed on the application of sourdough as a means to improve gluten-free bread quality., (Copyright © 2016 Elsevier B.V. All rights reserved.)

Dermatitis herpetiformis (DH) is a blistering skin disease, which is regarded as an extra-intestinal manifestation of coeliac disease. Refractory cases of coeliac disease, that do not respond to a gluten-free diet and which carry an increased risk of lymphoma, are well-known in coeliac disease. To determine whether refractory cases of DH with active rash and persistent small bowel atrophy occur we analysed our series of 403 patients with DH. Seven (1.7%) patients, who had been on a gluten-free diet for a mean of 16 years, but who still required dapsone to treat the symptoms of DH, were identified. Of these, one patient died from mucinous adenocarcinoma before re-examination. At re-examination skin immunoglobulin A (IgA) deposits were found in 5/6 refractory and 3/16 control DH patients with good dietary response. Small bowel mucosa was studied at re-examination from 5 refractory and 8 control DH patients and was normal in all 5 refractory and 7/8 control DH patients. One refractory DH patient died from adenocarcinoma, but no lymphoma developed in any of the patients. This study documents for the first time refractory DH, in which the rash is non-responsive to a gluten-free diet, but the small bowel mucosa heals. This differs from refractory coeliac disease, in which the small bowel mucosa does not heal on a gluten-free diet.

Background: Dermatitis herpetiformis (DH) is a cutaneous manifestation of celiac disease. Both conditions are treated with a restrictive life-long gluten-free diet (GFD). Treated celiac disease patients have been shown to have more severe gastrointestinal symptoms and inferior quality of life compared with healthy controls, but evidence regarding quality of life in DH is lacking., Objective: The aim was to evaluate whether long-term GFD-treated DH patients suffer from persistent gastrointestinal symptoms and if they experience a drawdown in quality of life., Methods: Gastrointestinal symptoms and quality of life were assessed in 78 long-term GFD-treated DH patients using the validated Gastrointestinal Symptom Rating Scale, Psychological General Well-Being and Short Form 36 Health Survey questionnaires. The findings were compared with 110 healthy controls, population-based reference values and 371 treated celiac disease controls., Results: The median age of the DH patients at the time of the study was 57 years, and 51 % were male. Significant differences in gastrointestinal symptoms or quality of life were not detected when treated DH patients were compared with healthy controls, but treated DH patients had less severe gastrointestinal symptoms and increased quality of life compared with celiac disease controls. Female DH patients had more severe gastrointestinal symptoms and reduced vitality compared with male DH patients. The presence of skin symptoms and the adherence to or duration of GFD did not have any influence on gastrointestinal symptoms or quality of life., Conclusion: We conclude that long-term GFD-treated DH patients do not suffer from the burden of dietary treatment and have a quality of life comparable to that of controls.

Bullous diseases may be rare; however, this does not preclude the clinician from being familiar with their manifestations and treatment. After ruling out infection, genetically inherited blistering diseases are more likely to be the cause of blistering or erosions in the neonatal period, whereas immunobullous diseases are more common in adults. Published literature on immunobullous disorders reflects information gleaned from case reports and open-label case series; prospective studies and evidence-based treatments are limited. Although there may be overlapping clinical features, significant clinical differences exist between adults and children. Evidence-based treatment guidelines are limited, and information from the adult literature cannot be readily generalized to the pediatric population. This paper reviews the approach to blistering conditions and the differences among bullous pemphigoid, linear immunoglobulin A disease, dermatitis herpetiformis, pemphigus foliaceus, pemphigus vulgaris, and paraneoplastic pemphigus in adult versus pediatric patients., (Copyright © 2015. Published by Elsevier Inc.)

Dermatitis herpetiformis (DH) is an itchy, blistering skin disease with sites of predilection at the elbows, knees and buttocks. Although DH is mostly asymptomatic, all patients exhibit small bowel villous atrophy or at least coeliac-type inflammatory changes. Deposition of immunoglobulin A (IgA) in the papillary dermis is a key diagnostic feature of DH. Epidermal transglutaminase (TG3) is the antigen for IgA deposited in the skin, and tissue transglutaminase (TG2) is the antigen for IgA deposited in the small bowel mucosa. Clinically silent, but immunologically active coeliac disease in the gut appears to result in IgA TG3 antibody complexes aggregated into DH skin. The prevalence of DH in northern Europe is high (30-75/100,000), but its incidence is decreasing, possibly due to increased recognition of subclinical coeliac disease. The rash and small bowel heal on a gluten-free diet, which is a life-long treatment. The risk of non-Hodgkin's lymphoma is increased, but in patients with DH who adhere strictly to a gluten-free diet long-term prognosis is excellent.

Objective: Dermatitis herpetiformis (DH) is a cutaneous form of celiac disease affecting ∼ 17% of celiac disease patients. The aim was to determine how often celiac disease precedes the development of DH, and what is the impact of gluten-free diet (GFD) in this phenotype change., Material and Methods: Our prospectively collected DH series from 1970 comprised 514 patients. We analyzed all DH patients who at least 2 years earlier had been diagnosed with celiac disease. DH diagnosis was confirmed by showing immunoglobulin A deposits in dermis. Serological and small bowel mucosal findings were analyzed, and the strictness of GFD treatment before and after the diagnosis of DH was evaluated., Results: Twenty (4%) DH patients had a prior diagnosis of celiac disease. The median time interval between celiac disease and DH detection was 9.5 years. Before DH appeared 4 patients had been on a normal gluten-containing diet, 10 had dietary lapses on a GFD, and 6 were on a strict GFD. Celiac autoantibodies were positive in 7 out of 19 patients, and 5 out of 7 undergoing small bowel biopsy had partial villous atrophy. Following DH diagnosis the rash was controlled after a median of 6 months on a strict GFD., Conclusions: Patients with celiac disease may develop DH by time. This is most often an indicator of poor adherence to GFD, and a rigorous dietary intervention is necessary. In the majority of cases, DH will be detected without prior celiac disease diagnosis, but the physicians should recognize this phenotype alteration.

Heat shock proteins (Hsp) are highly conserved immunomodulatory molecules upregulated when cells are exposed to stressful stimuli, such as inflammation. Their involvement in various autoimmune diseases, including autoimmune bullous diseases and celiac disease, has been increasingly recognized. To further study the role of Hsp in autoimmune bullous diseases, we have investigated for the first time the humoral autoimmune response to Hsp40, Hsp60, Hsp70, and Hsp90 in patients with dermatitis herpetiformis (DH; n = 26), bullous pemphigoid (BP; n = 23), and pemphigus vulgaris (PV; n = 16), the first representing a cutaneous manifestation of celiac disease. While in patients with active BP and PV, serum levels of autoantibodies against these Hsp did not differ from the corresponding age- and gender-matched healthy controls (n = 9-14); circulating autoantibodies against Hsp60, Hsp70, and Hsp90 were found to be increased at the active disease stage of DH. Further analysis of this latter patient subgroup showed that these anti-Hsp autoantibodies decreased in parallel with serum autoantibodies against epidermal and tissue transglutaminase during remission of skin lesions following a gluten-free diet, revealing significantly positive correlations. Although further studies on larger groups of patients will be needed to confirm the present data, our results support the notion that autoantibodies against Hsp60, Hsp70, and Hsp90 deserve attention in the study of the mechanisms that promote the development and maintenance of DH and possibly also the underlying celiac disease as well as potential novel disease biomarkers.

Background: Coeliac disease is an autoimmune disorder that is considered to affect approximately one in 100 people. In the UK, gluten-free (GF) foods can be prescribed by general practitioners (GPs) to treat this condition and there are national guidelines on the quantities of GF food an individual should receive on prescription. Information on actual prescribing behaviour by GPs, and how this compares with guideline recommendations, is scarce. The present study aimed to describe GPs prescribing practice of GF foods, within one locality in the UK, comparing this with national guidelines., Methods: A retrospective evaluation of GP electronic medical records for all patients with a gluten-sensitive enteropathy diagnosis and/or those prescribed GF food between April 2010 and March 2011 was carried out in 16 GP practices in the west of Scotland, serving a total of 85 667 patients., Results: Of 175 (0.18% of the total practice population) patients, 152 were identified with coeliac disease, eight with dermatitis herpetiformis and six with both conditions. A further nine patients received GF foods on prescriptions with no recorded diagnosis. There was a positive association between adherence to the prescribing guidelines and female sex (P < 0.0001) and (for those with a recorded diagnosis) increasing age (P = 0.001). There was no significant association between either socio-economic deprivation or co-morbidities and adherence to the prescribing guidelines., Conclusions: There was significant under prescribing of GF foods in those identified. Further research is required to establish whether these results are representative of wider practice in the UK., (© 2013 The Authors Journal of Human Nutrition and Dietetics © 2013 The British Dietetic Association Ltd.)

Background: Celiac disease can present with mild or nongastrointestinal symptoms, and may escape timely recognition. The treatment of celiac disease involves a gluten-free diet, which is complex and challenging., Objective: To evaluate clinical features and symptom recovery on a gluten-free diet in a Canadian adult celiac population., Methods: All adult members (n=10,693) of the two national celiac support organizations, the Canadian Celiac Association and Fondation québécoise de la maladie coeliaque, were surveyed using a questionnaire., Results: A total of 5912 individuals (≥18 years of age) with biopsy-confirmed celiac disease and⁄or dermatitis herpetiformis completed the survey. The female to male ratio was 3:1, and mean (± SD) age at diagnosis was 45.2 ± 16.4 years. Mean time to diagnosis after onset of symptoms was 12.0 ± 14.4 years. Abdominal pain and bloating (84.9%), extreme weakness⁄tiredness (74.2%), diarrhea (71.7%) and anemia (67.8%) were the most commonly reported symptoms at the time of diagnosis. Many respondents continued to experience symptoms after being on a gluten-free diet for >5 years. Sex differences were reported in clinical features before diagnosis, recovery after being on gluten-free diet and perceived quality of life, with women experiencing more difficulties than men., Conclusions: Delays in diagnosis of celiac disease in Canada remain unacceptably long despite wider availability of serological screening tests. Many patients report continuing symptoms despite adhering to a gluten-free diet for >5 years, with women experiencing more symptoms and a lower recovery rate than men. Awareness of celiac disease needs improvement, and follow-up with a physician and a dietitian is essential for all patients with celiac disease.

Aim: The aim of this paper to report the main clinical and immunopathological findings of our case series of 159 patients with dermatitis herpetiformis (DH)., Methods: All DH patients that were diagnosed from 1995 to 2012 at the Section of Dermatology of the University of Florence were included in the study. Clinical data were collected for each patient. Moreover, histopathological examination on both the skin and the small bowel, direct immunofluorescence on perilesional skin as well as the search for anti-endomysium and anti-tissue transglutaminsase antibodies (tTG) were performed., Results: A total of 159 patients with a male predominance were enrolled. About 36% of the patients were below the age of 20. The most frequent clinical features seen in our DH patients were represented by figurate erythema, wheals, papules and scratching lesions, while the knees, elbows and buttocks were the most commonly involved sites. All the 22 patients that underwent a bowel biopsy showed the typical alterations found in celiac disease. Moreover, 100% of the patients showed granular IgA deposits at the papillary tips. Finally, anti-endomysium and anti-tTG antibodies were present in 90% and 96% of the patients, respectively., Conclusion: We reported one of the largest case series of patients with DH from a single center. Our study confirmed most of the data from the Literature, and in particular the association of DH to histologically proven CD in all the biopsied cases. Another interesting finding of our study is the high prevalence of DH within pediatric patients, that is usually underreported.

Objective: Strict adherence to a gluten-free diet is the only treatment for coeliac disease. The gluten-free diet is complex, costly and impacts on all activities involving food, making it difficult to maintain for a lifetime. The purpose of this cross-sectional study was to evaluate the difficulties experienced, the strategies used and the emotional impact of following a gluten-free diet among Canadians with coeliac disease., Methods: A questionnaire was mailed to all members (n = 10 693) of both the Canadian Celiac Association and the Fondation québécoise de la maladie cœliaque in 2008., Results: The overall response rate was 72%. Results are presented for the 5912 respondents (≥18 years) reporting biopsy-confirmed coeliac disease and/or dermatitis herpetiformis. Two-thirds never intentionally consumed gluten. Women reported significantly greater emotional responses to a gluten-free diet but, with time, were more accepting of it than men. Difficulties and negative emotions were experienced less frequently by those on the diet for >5 years, although food labelling and eating away from home remained very problematic. Frustration and isolation because of the diet were the most common negative emotions experienced., Conclusions: The present study quantifies the difficulties experienced, the strategies used and the emotional impact of following a gluten-free diet. It highlights the need to improve the training and education of dietitians, other health providers and the food service industry workers about coeliac disease and a gluten-free diet, with the aim of better helping individuals improve their adherence to a gluten-free diet and their quality of life., (© 2012 Canadian Celiac Association and Food Directorate Health Canada Journal of Human Nutrition and Dietetics © 2012 The British Dietetic Association Ltd.)

A gluten-free diet (GFD) is recommended for all patients with coeliac disease (CD). The spectrum of gluten-related disorders in the early 1980s was simple: CD and dermatitis herpetiformis. In the last few years, wheat allergy, gluten ataxia and noncoeliac gluten sensitivity have become new gluten-related topics. Adherence to GFDs in CD is limited and factors influencing adherence are poorly understood. Noncoeliac gluten sensitivity has stimulated the GFD food industry not only in Australia but all over the world. This article provides an overview of GFD in daily practice., (Copyright © 2013 S. Karger AG, Basel.)

We present a 15-year-old female patient with a 6-month history of recurrent painful petechiae on the fingers and feet. Trauma or pressure were denied, but she reported recurrent tonsillitis and urinary tract infections and a single event of bilateral scotoma. Extensive investigations (e.g., echocardiography) for a suspected diagnosis of septic emboli were unremarkable. Routine histopathology, direct and indirect immunofluorescence, and esophagogastroduodenoscopy led to the diagnosis of dermatitis herpetiformis. The therapeutic strategy comprised gluten-free diet and dapsone to alleviate the symptoms. Dermatitis herpetiformis should be included in the differential diagnosis of palmar or plantar petechiae, especially when occurring in children or young adults., (© 2011 Wiley Periodicals, Inc.)

Dermatitis herpetiformis (DH) is a chronic, polymorphic, pruritic skin disease that develops mostly in patients with latent gluten-sensitive enteropathy. DH patients usually present with skin manifestations only and are not aware of the underlying small-bowel problems. Owing to the granular immunoglobulin (Ig) A deposition at the tips of the papillary dermis and to the subepidermal blister formation associated with neutrophilic accumulations underlying the basement membrane, DH is considered to be an autoimmune blistering disease. Contrary to the other bullous disorders, DH patients have no circulating autoantibodies binding to the cutaneous basement membrane components or to other adherent structures of the skin, but they have gluten-induced IgA autoantibodies against transglutaminase (TG) 2 and TG3. The serum IgA against tissue TG2 is a most specific and sensitive serologic marker of gluten-sensitive enteropathy and is equivalent to the perviously described IgA endomysium antibodies. DH could be a cutaneous IgA-epidermal TG3 immunocomplex disease, developing only in a few patients with gluten-sensitive enteropathy as a second gluten-dependent disease. The main treatment of DH today is a strict, life-long gluten-free diet. Untreated DH patients should be regularly monitored for malabsorption and lymphomas. Associated autoimmune diseases are more common among DH patients. Family screening for gluten sensitivity is also strongly suggested., (Copyright © 2012. Published by Elsevier Inc.)

As the gluten-free diet (GFD) gains in popularity with the general public, health practitioners are beginning to question its real health benefits. For those patients with celiac disease (CD), the GFD is considered medical nutrition therapy, as well as the only proven treatment that results in improvements in symptomatology and small bowel histology. Those with wheat allergy also benefit from the GFD, although these patients often do not need to restrict rye, barley, and oats from their diet. Gluten sensitivity is a controversial subject, where patients who have neither CD nor wheat allergy have varying degrees of symptomatic improvement on the GFD. Conditions in this category include dermatitis herpetiformis (DH), irritable bowel syndrome (IBS), and neurologic diseases such as gluten-sensitive ataxia and autism. It is important for patients and healthcare practitioners to understand the differences between these conditions, even though they may all respond to a GFD. Patients with CD can experience comorbid nutrition deficiencies and are at higher risk for the development of cancers and other autoimmune conditions. Those with wheat allergy and gluten sensitivity are thought not to be at higher risk for these complications. Defining the symptoms and biochemical markers for gluten-sensitive conditions is an important area for future investigations, and high-quality, large-scale randomized trials are needed to prove the true benefits of the GFD in this evolving field.

A 10-year-old girl presented with chronic, itchy papulo-vesiculous skin lesions. Diagnosis of dermatitis herpetiformis was established based on elevated IgA endomysium and transglutaminase type 2 antibodies, and histological findings on skin biopsy. Dermatitis herpetiformis is the cutaneous expression of celiac disease. Treatment consists of gluten-free diet and diafenylsulfon.

Background: Dermatitis herpetiformis is an autoimmune disease typically characterized by pruritic vesicles located on the extensor surfaces. Classic disease consists of neutrophils in the dermal papillae. Additional histopathologic findings include fibrin deposition and edema within the dermal papillae. Subepidermal vesicles also may be present. Direct immunofluorescence demonstrates granular IgA in the dermal papillae., Observations: A 58-year-old man with tender and pruritic erythematous macules and papules ranging from 2 to 6 mm in diameter had bilateral knee, elbow, forearm, scalp, and neck involvement. Petechiae also were present on the hands, thigh, knee, and ankle. A biopsy specimen initially demonstrated leukocytoclastic vasculitis. The results of workup for systemic vasculitis were negative. Subsequent biopsy specimens and direct immunofluorescence showed histologic evidence of dermatitis herpetiformis and leukocytoclastic vasculitis in the setting of an elevated serum IgA antitissue transglutaminase level. Marked improvement of the lesions was observed with a reduction of gluten in the patient's diet., Conclusions: Physicians should consider the possibility of dermatitis herpetiformis in patients with petechiae and leukocytoclastic vasculitis because leukocytoclastic vasculitis may be a prominent feature of dermatitis herpetiformis.

The major treatment strategies for DH are gluten restriction or medical treatment with sulfones. Control of the cutaneous manifestations, but not the gastrointestinal changes, is rapid with dapsone. In addition to control of the cutaneous signs and symptoms of DH, dietary gluten restriction also induces improvement of gastrointestinal morphology and is possibly protective against the development of lymphoma., (Copyright © 2011 Elsevier Inc. All rights reserved.)

The prompt recognition of the clinical features of dermatitis herpetiformis (DH) is important, but securing a definitive diagnosis requires further work-up. Recent advances in understanding of the immunologic basis for DH have led to the development and wider availability of serologic testing, which is rapidly becoming an essential part of the diagnosis and management of DH. Part II of this series will detail the diagnosis, treatment, and follow-up for patients with DH, and will particularly focus on recent advances in the field., (Copyright © 2010 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.)

A gluten-free diet (GFD) is commonly recognized as the treatment for celiac disease. It also has been investigated as a treatment option for other medical conditions, including dermatitis herpetiformis, irritable bowel syndrome, neurologic disorders, rheumatoid arthritis, diabetes mellitus, and HIV-associated enteropathy. The strength of the evidence for the use of a GFD in these nonceliac diseases varies, and future research may better define the benefits of a GFD for those conditions with weak existing evidence.

Background: Dermatitis herpetiformis (DH) is a rare auto-immune bullous disease characterized by its almost constant association to gluten sensitivity., Objective: Review of literature about epidemiology, physiopathology, clinical data and treatment of DH., Methods: Research on Medline and Embase database without any time limit until April 2010. Because of the lack of randomized therapeutic trials in DH, retrospective series and case reports have been analyzed., Results: DH is related to auto-antibodies against epidermal transglutaminase, which belongs to the same family as tissue transglutaminase, the auto-antigen of celiac disease. Physiopathology is complex, occurring in HLA DQ2 or DQ8 predisposed patients, and implies gluten, immunological reaction in the intestinal wall then in the skin. DH and celiac disease may be encountered in the same family. DH is characterized by a very pruritic microvesicular eruption typically located on elbows, knees and buttocks. Digestive manifestations of celiac disease occur in 15% of cases. Direct immunofluorescence is necessary to confirm the diagnosis, showing granular IgA±C3 deposits in the papillary dermis. Circulating IgA and IgG antiendomysium and antitransglutaminase antibodies are detected in almost all patients at the acute phase and follow the clinical course of the disease. Gastro-intestinal endoscopy with multiple duodenal biopsies shows partial or complete villous atrophy in two thirds of cases, intraepithelial lymphocyte infiltrate in the other cases. Other auto-immune diseases may be associated in 10-20% of cases. The main long-term risk is the occurrence of T or B nodal or intestinal tract lymphoma in 2% of cases (relative risk close to 6 in several studies, but not admitted by all authors), especially if adherence to gluten-free diet is not strict. Treatment is based on dapsone, which is quickly efficient on cutaneous manifestations, but not on the digestive involvement and on strict and definitive gluten-free diet, which cures villous atrophy and reduces the risk of lymphoma., Conclusion: DH is associated to a gluten enteropathy and its physiopathology is better known. Even if the risk of secondary lymphoma seems little, most of the authors recommend a definitive gluten-free diet., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

Objectives: To determine the percentage of patients with dermatitis herpetiformis (DH) who experience at least 2 years of remission and to identify factors associated with DH remission., Design: Retrospective cohort study., Setting: National Institutes of Health (NIH)., Patients: Patients seen at the NIH during the 1972-2010 period who had clinical findings consistent with DH, whose normal skin showed the presence of granular IgA deposits at the dermoepidermal junction on direct immunofluorescence (DIF) examination, whose age of disease onset was known, who had DH for at least 2 years, and who were followed up for at least 3 years after the initial NIH visit., Main Outcome Measure: Remission, defined as absence of skin lesions and symptoms of DH for more than 2 years while not taking sulfones (dapsone or sulfoxone), sulfapyridine, anti-tumor necrosis factor agents, or oral steroids and not adhering to a gluten-free diet., Results: Among 86 patients, in 10 (12%) the disease underwent remission (95% confidence interval, 6%-20%). Factors associated with DH remission included DH age of onset at 39 years or older vs onset at ages 8 to 38 years (unadjusted P = .02; adjusted P = .07) and DH onset year between 1960 and 1972 vs onset between 1935 and 1959 or after 1972 (P = .02 for global comparison of 4 onset-year groups)., Conclusions: Dermatitis herpetiformis can go into remission. Clinicians should attempt to wean patients with well-controlled DH from a gluten-free diet and/or use of sulfones or other therapies to determine if the DH might have remitted. Our findings provide insight into the pathogenesis and course of this disease and may serve to guide long-term management of patients with DH.

Autoimmune and nonautoimmune bullous diseases can both be associated with significant morbidity and mortality. Although our understanding of the pathogenic mechanisms of these diseases has increased tremendously, there is still much to learn about the various factors affecting their onset, course, and therapy. In recent years, increasing information has been published about the effect of vitamins, minerals, and other nutrients on bullous skin diseases. Some factors are believed to be inducers (thiol and phenol-containing foods in pemphigus), whereas others are believed to be protective (antioxidants in cutaneous porphyrias). This contribution reviews the evidence in the literature of the role of various dietary factors in bullous diseases, including the nonautoimmune and the deficiency dermatoses. Additional studies and new investigations are needed to provide a better understanding of the specific associations of dietary factors with bullous diseases and better management for patients affected by these conditions., (Copyright © 2010. Published by Elsevier Inc.)

Celiac disease is an immune-mediated disease, triggered in genetically susceptible individuals by ingested gluten from wheat, rye, barley, and other closely related cereal grains. The only treatment for celiac disease is a strict gluten-free diet for life. This paper presents a systematic review of the scientific literature on the safety of pure oats for individuals with celiac disease, which historically has been subject to debate. Limitations identified within the scientific database include: limited data on long-term consumption, limited numbers of participants in challenge studies, and limited reporting about the reasons for withdrawals from study protocols. Furthermore, some evidence suggests that a small number of individuals with celiac disease may be intolerant to pure oats and some evidence from in vitro studies suggests that an immunological response to oat avenins can occur in the absence of clinical manifestations of celiac disease as well as suggesting that oat cultivars vary in toxicity. Based on the majority of the evidence provided in the scientific database, and despite the limitations, Health Canada and the Canadian Celiac Association (CCA) concluded that the majority of people with celiac disease can tolerate moderate amounts of pure oats. The incorporation of oats into a gluten-free diet provides high fiber and vitamin B content, increased palatability, and beneficial effects on cardiovascular health. However, it is recommended that individuals with celiac disease should have both initial and long-term assessments by a health professional when introducing pure oats into a gluten-free diet.