Search

Background: Brief erythematous-papular skin rashes suggest the diagnosis of urticaria; However, it may be another type of dermatitis, and complementary examinations must be carried out to establish its diagnosis., Case Report: 53-year-old female patient, diagnosed in 2016 with diffuse large B cell lymphoma, in complete remission. Since 2010, he has had episodes of erythematous-papular lesions lasting 24-36 hours. He received antihistamines, corticosteroids and omalizumab without clinical improvement. The ANA determination was positive (1/320), nuclear mitotic pattern. The skin biopsy was compatible with dermatitis herpetiformis. The study of celiac and locus antibodies showed positivity for HLA-DQ2 and DQ2.5 in heterozygosity. The diagnosis of dermatitis herpetiformis was established. Treatment consisted of a gluten-free diet and prescription of dapsone, with satisfactory results., Conclusion: It is important to establish the differential diagnosis of patients with chronic urticaria who do not respond to the reference treatment, in addition to carrying out a thorough clinical examination and physical examination before starting treatment and relying on a multidisciplinary team to establish an accurate diagnosis and treatment. appropriate. Due to the side effects of dapsone, subsequent follow-up of patients is essential.

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.

Dermatitis herpetiformis is a cutaneous form of celiac disease manifesting as an itching rash typically on the elbows, knees and buttocks. It is driven by the ingestion of gluten-containing cereals and characterized by granular deposits of immunoglobulin A in the papillary dermis. These antibodies target transglutaminase (TG) 3 and in the majority of patients they are also found in circulation. The circulating antibodies disappear and skin symptoms resolve as a result of gluten-free diet but the cutaneous anti-TG3 IgA deposits may persist for several years. In dermatitis herpetiformis, plasma cells secreting antibodies against TG3 are located in the intestinal mucosa similarly to those producing TG2 antibodies characteristic for celiac disease. In fact, both TG2- and TG3-specific plasma cells and gluten responsive T cells are found in dermatitis herpetiformis patients but the interplay between these cell populations is unknown. The small bowel mucosal damage in celiac disease is believed to be mediated by co-operation of cytotoxic intraepithelial T cells and the inflammatory milieu contributed by gluten-reactive CD4+ T cells, whereas the skin lesions in dermatitis herpetiformis appear to be devoid of gluten reactive T cells. Thus, how celiac disease-type intestinal T and B cell responses develop into an autoimmune condition affecting the skin is still incompletely understood. Finally, the skin and small bowel lesions may reappear upon reintroduction of gluten in patients treated with gluten-free diet but virtually nothing is known about the long-lived B cell and memory T cell populations activating in response to dietary gluten in dermatitis herpetiformis., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Kemppainen, Salmi and Lindfors.)

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 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 is a cutaneous manifestation of celiac disease that classically presents as a symmetric pruritic vesicular eruption on extensor surfaces. Typical locations include elbows, knees, and buttocks. Facial involvement has been reported rarely. Here, we report a case of a 44-year-old woman with dermatitis herpetiformis presenting as pruritic vesicles on the face that had previously been misdiagnosed as allergic contact dermatitis. Diagnosis was confirmed with direct immunofluorescence demonstrating granular IgA in the papillary dermis. This eruption cleared with topical dapsone 5% gel and a gluten-free diet. We report this case to raise awareness of facial involvement in dermatitis herpetiformis as well as the possibility of topical dapsone as a therapeutic option.

The skin is the largest organ of the body, at the boundary with the outside environment. Primarily, it provides a physical and chemical barrier against external insults, but it can act also as immune organ because it contains a whole host of immune-competent cells of both the innate and the adaptive immune systems, which cooperate in eliminating invading pathogens following tissue injury. On the other hand, improper skin immune responses lead to autoimmune skin diseases (AISD), such as pemphigus, bullous pemphigoid, vitiligo, and alopecia. Although the interplay among genetic, epigenetic, and environmental factors has been shown to play a major role in AISD etiology and progression, the molecular mechanisms underlying disease development are far from being fully elucidated. In this context, epidemiological studies aimed at defining the association of different AISD with other autoimmune pathologies revealed possible shared molecular mechanism(s) responsible for disease progression. In particular, over the last decades, a number of reports have highlighted a significant association between thyroid diseases (TD), mainly autoimmune ones (AITD), and AISD. Here, we will recapitulate the epidemiology, clinical manifestations, and pathogenesis of the main AISD, and we will summarize the epidemiological evidence showing the associations with TD as well as possible molecular mechanism(s) underlying TD and AISD pathological manifestations.

Dermatitis herpetiformis (DH) is a cutaneous manifestation of coeliac disease. At diagnosis, the majority of patients have villous atrophy in the small bowel mucosa. The objective of this study was to investigate whether the presence or absence of villous atrophy at diagnosis affects the long-term prognosis of DH. Data were gathered from the patient records of 352 DH and 248 coeliac disease patients, and follow-up data via questionnaires from 181 DH and 128 coeliac disease patients on a gluten-free diet (GFD). Of the DH patients, 72% had villous atrophy when DH was diagnosed, and these patients were significantly younger at diagnosis compared to those with normal small bowel mucosa (37 vs. 54 years, p < 0.001). Clinical recovery on a GFD did not differ significantly between the DH groups, nor did current adherence to a GFD, the presence of long-term illnesses, coeliac disease-related complications or gastrointestinal symptoms, or quality of life. By contrast, the coeliac disease controls had more often osteopenia/osteoporosis, thyroid diseases, malignancies and current gastrointestinal symptoms compared to the DH patients. In conclusion, villous atrophy at the time of DH diagnosis does not have an impact on the clinical recovery or long-term general health of DH patients., Competing Interests: The authors report no conflicts of interest.

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 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.

Researches on DH have shown that it is not just a bullous skin disease, but a cutaneous-intestinal disorder caused by hypersensitivity to gluten. Exposure to gluten is the starting point of an inflammatory cascade capable of forming autoantibodies that are brought to the skin, where they are deposited, culminating in the formation of skin lesions. These lesions are vesico-bullous, pruritic, and localized especially on elbows, knees and buttocks, although atypical presentations can occur. Immunofluorescence of perilesional area is considered the gold standard for diagnosis, but serological tests help in cases where it is negative. Patients who follow gluten-free diets have better control of symptoms on the skin and intestine, as well as lower risks of progression to lymphoma. Dapsone remains the main drug for treatment, but it requires monitoring of possible side effects, some potentially lethal.

Background: Dermatitis herpetiformis (DH) is a skin manifestation of celiac disease (CD), and is often the presenting and only complaint. There are few data comparing those with DH who present only with DH and those with DH who present mainly due to CD., Objective: We compared the prevalence of features usually associated with CD in those who presented with DH with patients in whom DH was part of a typical CD presentation., Methods: A cross-sectional study of a prospectively maintained database of 1,050 patients with CD was analyzed. Only biopsy-diagnosed patients were analyzed for small bowel findings. All patients were included in the analysis of autoimmune diseases and lymphoma incidence. Small bowel biopsies were classified into mild and severe., Results: The prevalence of villous atrophy was significantly higher in the patients who presented with CD than in those who presented with DH alone (61.8 vs. 12.5%; p = 0.005). However, the prevalence of nutritional deficiencies, autoimmune diseases, and lymphoma occurred at a similar rate in patients with DH and patients with CD without DH., Conclusions: Patients who present with CD and concurrent DH are more likely to have more severe pathology than those with predominantly DH, although nutritional deficiencies are similar between the two groups. It is important to screen for nutritional deficiencies in patients with DH, irrespective of the presence of typical CD manifestations.

The skin is often a window to systemic disease that is available to the trained eye of the dermatologist. Herein, we focus on four dermatoses with associated systemic conditions of interest: scleromyxedema and monoclonal gammopathy, nephrogenic systemic fibrosis in the setting of renal insufficiency, dermatitis herpetiformis and celiac disease, and psoriasis as a risk factor for cardiovascular disease. Dermatologists can play a crucial role in recognizing the cutaneous manifestations linked with these conditions. Identifying the related underlying disorder will contribute to appropriate diagnosis and improved management.

Background: Celiac disease is classically manifested in the gastrointestinal (GI) tract but extraintestinal symptoms, such as dermatitis herpetiformis (DH), are also common. Besides several well-known shared genetic risk factors and an environmental trigger, gliadin, factors determining the clinical outcome of the disease are not known. In this study, the role of duodenal microbiota in the celiac disease outcome was studied by analyzing mucosa-associated microbiota in celiac disease patients with a variety of intestinal and extraintestinal symptoms., Methods: Microbiota in duodenal biopsy samples obtained from 33 patients with celiac disease with GI, DH, anemia, or mixed symptoms, as well as screen-detected asymptomatic celiac disease and 18 control subjects were analyzed using PCR denaturing gradient gel electrophoresis and a subset of samples additionally by the 16S ribosomal RNA gene sequencing., Results: The composition and diversity of mucosal microbiota was associated with the manifestation of celiac disease when analyzed using PCR denaturing gradient gel electrophoresis and the 16S ribosomal RNA gene sequencing. The patients with celiac disease with GI symptoms or anemia had lower microbial diversity than those with DH. Moreover, the patients with GI symptoms had different intestinal microbiota composition and structure, dominated by Proteobacteria, in comparison to those with DH or control subjects (patients with dyspepsia). The relatively similar intestinal microbiota composition in the control subjects and those with DH was characterized by the high abundance of Firmicutes., Conclusions: The two common outcomes of celiac disease, classical GI and extraintestinal manifestations, had marked differences on the diversity and composition of intestinal microbiota. This association suggested that intestinal microbiota may have a role in the manifestation of the disease.

Objective: The literature suggests a lack of consensus on the use of terms related to coeliac disease (CD) and gluten., Design: A multidisciplinary task force of 16 physicians from seven countries used the electronic database PubMed to review the literature for CD-related terms up to January 2011. Teams of physicians then suggested a definition for each term, followed by feedback of these definitions through a web survey on definitions, discussions during a meeting in Oslo and phone conferences. In addition to 'CD', the following descriptors of CD were evaluated (in alphabetical order): asymptomatic, atypical, classical, latent, non-classical, overt, paediatric classical, potential, refractory, silent, subclinical, symptomatic, typical, CD serology, CD autoimmunity, genetically at risk of CD, dermatitis herpetiformis, gluten, gluten ataxia, gluten intolerance, gluten sensitivity and gliadin-specific antibodies., Results: CD was defined as 'a chronic small intestinal immune-mediated enteropathy precipitated by exposure to dietary gluten in genetically predisposed individuals'. Classical CD was defined as 'CD presenting with signs and symptoms of malabsorption. Diarrhoea, steatorrhoea, weight loss or growth failure is required.' 'Gluten-related disorders' is the suggested umbrella term for all diseases triggered by gluten and the term gluten intolerance should not to be used. Other definitions are presented in the paper., Conclusion: This paper presents the Oslo definitions for CD-related terms.

Background: Dermatitis herpetiformis (DH) is an extra-intestinal manifestation of coeliac disease and most patients adhere to a life-long gluten free diet (GFD). Increased mortality rates have been reported in coeliac disease but knowledge in DH is scanty., Objectives: To survey the mortality rate and causes of death in a large cohort of patients with DH., Material and Methods: Patients with DH (n = 476 consecutive patients) diagnosed from 1970 onwards at the Tampere University Hospital were analysed for causes of death during 1971-2010. A questionnaire survey on key aspects of health behaviour was performed in patients with DH and comparisons were made with the Finnish population., Results: The total number of deaths during 9079 person years followed up was 77 whereas 110 were expected. The standardized mortality rate (SMR) for all causes of death was significantly reduced, being 0·70 (95% CI 0·55-0·87), and similar in both sexes. The SMR was equal in the patients with DH with (0·73) and without (0·77) small bowel villous atrophy. The SMR was significantly reduced (0·38) for deaths due to cerebrovascular diseases. The SMR due to lymphoproliferative malignancies was significantly increased (6·86) in the first 5 years of follow-up but not thereafter. The questionnaire survey documented that 97·7% of the patients with DH adhered to a GFD. The patients reported significantly less hypercholesterolaemia and there were fewer current and past smokers compared with the age- and sex-matched control population., Conclusions: The present long-term follow-up study of DH documented significantly reduced all-cause and cerebrovascular disease mortality. Strict adherence to a GFD, less smoking and hypercholesterolaemia may play a role in the observed health benefit., (©The Authors BJD © 2012 British Association of Dermatologists.)

Background: Dermatitis herpetiformis (DH) is a cutaneous manifestation of celiac disease (CD), the latter being identified by circulating autoantibodies against native gliadin (nGli), tissue transglutaminase (tTG), endomysium, or a combination of these. Recently, a novel serologic assay using deamidated gliadin-analogous fusion peptides (GAF3X) showed high diagnostic sensitivity in patients with CD., Objective: The aim of this study was to explore the anti-GAF3X enzyme-linked immunosorbent assay (ELISA) and to compare it with a panel of classic CD-related serologic tests in patients with DH., Methods: Antibodies against nGli, GAF3X, and tTG were determined by separate ELISA tests and against endomysium by indirect immunofluorescence microscopy in 45 patients with DH and 52 control patients (30 patients with bullous pemphigoid and 22 patients with linear IgA disease). A total of 24 patients with DH underwent intestinal biopsies to confirm underlying CD., Results: Antibodies to nGli were detected in 26 (58%) (IgA) and 24 (53%) (IgG), to GAF3X in 38 (84%) (IgA) and 36 (80%) (IgG), to tTG in 35 (78%) (IgA), and to endomysium in 32 (71%) (IgA) patients with DH. Combined testing of IgA and IgG antibodies to GAF3X detected 7 of 10 (70%) IgA-anti-tTG-negative patients with DH and together with the IgA-anti-tTG ELISA showed the highest serologic hit rate (93%) for CD., Limitations: Intestinal biopsies were not performed in all patients with DH., Conclusion: The novel anti-GAF3X ELISA shows a higher sensitivity to detect CD-associated autoantibodies in patients with DH compared with tests using nGli, tTG, or endomysium as substrates., (Copyright © 2011 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.)

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.

Dermatitis herpetiformis (DH) is characterized by chronic, itching papules, seropapules, small vesicles and, exceptionally, large blisters. The distribution of these polymorphic symptoms around the elbow, knee, buttock, and back is suggestive of the diagnosis. DH is further confirmed by the accumulation of granulocytes at the papillary dermis, resulting in a subepidermal split formation and by the presence of a unique, granular IgA precipitate in the uppermost dermis. Prognosis is predominantly determined by other autoimmune pathologies, malabsorption, or very rarely by lymphomas. Some of these diseases can be prevented by an early-onset, strict gluten-free diet, which is therefore the suggested treatment option., (Copyright © 2011. Published by Elsevier Inc.)

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.)

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.)

Background: Dermatitis herpetiformis is a chronic severely pruritic dermatosis. It is a cutaneous manifestation of celiac disease. The aim of our study was to collect clinical, histological and immunopathological data on patients who were treated in the University Departments of Dermatology in Würzburg and Lübeck from 1996 to 2008., Patients and Methods: We retrospectively analyzed 32 patients. Only patients with positive findings on direct immunofluorescence microscopy were included in this study., Results: All patients demonstrated skin lesions in the predilection areas of knees, elbows, gluteal region and scalp. The male to female ratio was 1.5 : 1 and the average age was 43 years. The interval between the first symptoms and diagnosis ranged from 6 weeks to 20 years. Direct immunofluorescence microscopy showed that granular IgA deposits were more often found continuously along the dermal-epidermal junction rather than focally in the tips of the dermal papillae. Results of small intestinal biopsies were available from 29 patients and confirmed the presence of celiac disease in all cases. None of the patients reported gastrointestinal symptoms. IgA antibodies against tissue transglutaminase and epidermal transglutaminase were found in 88% and 94% of patient sera, respectively., Conclusions: The detection of IgA autoantibodies against epidermal transglutaminase is the most sensitive serological test in the diagnosis of dermatitis herpetiformis. Our observations confirm that patients with dermatitis herpetiformis usually do not demonstrate apparent gastrointestinal symptoms.

Objective: Coeliac disease (CD) is associated with immune-mediated skin diseases such as dermatitis herpetiformis and others. The objective of the study was to investigate the relation of body mass index (BMI), as an index of absorptive status, with the prevalence of skin diseases in adults with untreated CD., Methods: Anthropometry, gastro-intestinal symptoms, nutritional indices and immune-mediated skin diseases (dermatitis herpetiformis, psoriasis, aphthosis and alopecia) at diagnosis were analysed., Results: 223 men and 924 women with untreated CD (aged 20-60 years) were included, the commonest skin disease was dermatitis herpetiformis (18.4 and 6.9%, respectively), the rarest one was alopecia (1.8 and 2.1%). The BMI was positively associated with male gender, age at diagnosis and nutritional indices, negatively with diarrhoea and dyspepsia (p < 0.001). A BMI difference of 3.5 (1 standard deviation) was related to an excess prevalence of dermatitis herpetiformis (odds ratio, OR = 1.46, 95% confidence interval, CI = 1.23-1.72) and of psoriasis (OR = 1.40, 95% CI = 1.10-1.79) but not of other immunological disorders. Findings were similar in analyses by gender or age group and controlled for gender and age. The relation of BMI to dermatitis herpetiformis was linear over the whole BMI range, also excluding overweight patients. The relation of BMI to psoriasis was flat for low-to-normal BMI and explained only by overweight patients., Conclusion: In CD at diagnosis, the BMI is positively related to the prevalence of dermatitis herpetiformis and psoriasis, not to that of other immune-mediated skin diseases., (Copyright 2009 S. Karger AG, Basel.)

Coeliac disease may have an ancient history dating back to the 1st and 2nd centuries AD. The first clear description was given by Samuel Gee in 1888. He suggested that dietary treatment might be of benefit. In the early 20th century various diets were tried, with some success, but without clear recognition of the toxic components. The doctoral thesis of Wim Dicke of 1950 established that exclusion of wheat, rye and oats from the diet led to dramatic improvement. The toxicity was shown to be a protein component, referred to as gluten. Dicke's colleagues, Weijers and Van de Kamer, showed that measurement of stool fat reflected the clinical condition. Early studies were in children but stool fat measurements documented that the condition could be recognised in adults. Histological abnormalities of the lining of the small intestine were demonstrated beyond doubt by Paulley in 1954 and techniques of per-oral biopsy described by Royer in 1955 and Shiner in 1956 afforded reliable diagnosis. Concurrence in monozygotic twins suggested a genetic component, confirmed by studies of HLA antigens. Additional, non-genetic factors seem likely. Circulating antibodies suggest an immunological mechanism of damage and provide non-invasive screening tests. Lymphoma, adenocarcinoma and ulceration of the small intestine and a range of immunological disorders are associated. A relationship with dermatitis herpetiformis was suggested by Samman in 1955 and established by Shuster and Marks in 1965 and 1968. The Coeliac Society (now Coeliac UK) was founded in 1968 and similar societies now exist across the world. They provide an extremely valuable service. Present problems include definition of the tolerated levels of gluten, whether oats are toxic for some or all coeliacs and the likelihood that the condition is relatively common and frequently without classical symptoms. Hope for the future is that more convenient methods of treatment will follow better understanding., (2008 S. Karger AG, Basel.)

Celiac disease (CD) is a common autoimmune disorder, induced by the intake of gluten proteins present in wheat, barley and rye. Contrary to common belief, this disorder is a protean systemic disease, rather than merely a pure digestive alteration. CD is closely associated with genes that code HLA-II antigens, mainly of DQ2 and DQ8 classes. Previously, it was considered to be a rare childhood disorder, but is actually considered a frequent condition, present at any age, which may have multiple complications. Tissue transglutaminase-2 (tTG), appears to be an important component of this disease, both, in its pathogenesis and diagnosis. Active CD is characterized by intestinal and/or extra-intestinal symptoms, villous atrophy and crypt hyperplasia, and strongly positive tTG auto-antibodies. The duodenal biopsy is considered to be the "gold standard" for diagnosis, but its practice has significant limitations in its interpretation, especially in adults. Occasionally, it results in a false-negative because of patchy mucosal changes and the presence of mucosal villous atrophy is often more severe in the proximal jejunum, usually not reached by endoscopic biopsies. CD is associated with increased rates of several diseases, such as iron deficiency anemia, osteoporosis, dermatitis herpetiformis, several neurologic and endocrine diseases, persistent chronic hypertransami-nasemia of unknown origin, various types of cancer and other autoimmune disorders. Treatment of CD dictates a strict, life-long gluten-free diet, which results in remission for most individuals, although its effect on some associated extraintestinal manifestations remains to be established.

Celiac disease is a genetically determined bowel disease also influenced by exogenous factors in which exposure to grain components triggers a chronic immune response with intestinal symptoms. Dermatitis herpetiformis represents the cutaneous manifestation of celiac disease. While intense pruritus is the characteristic symptom, clinical signs can be highly variable, ranging from grouped papulovesicles with excoriations or eczema-like lesions to minimal variants of discrete erythema and digital purpura. Diagnosis depends on direct fluorescence studies of perilesional skin displaying granular IgA deposits in dermal papillae. Suspecting and then searching for dermatitis herpetiformis is often clinically challenging, as the disease is a true chameleon with many clinical faces. Dapsone therapy alleviates the cutaneous symptoms and signs, but does not prevent the systemic complications of celiac disease; thus, strict adherence to a gluten-free diet is strongly advisable.

Celiac disease is an autoimmune disorder that occurs in genetically predisposed individuals as the result of an immune response to gluten. It is present in approximately 1% of the population. Diarrhea has become a less common mode of presentation (<50% of cases) than it once was. Other presentations include iron-deficiency anemia, osteoporosis, dermatitis herpetiforme, and neurologic disorders, mainly peripheral neuropathy and ataxia. Arthritis is commonly found in patients with celiac disease when systematically sought. Overall, autoimmune diseases occur more frequently (three to ten times more) in those with celiac disease than in the general population. A gluten-free diet is the standard of treatment, although its effect on some of the extraintestinal manifestations remains to be determined.

A systematic review of the literature related to the inclusion of oats in the gluten-free diet for patients with coeliac disease to assess whether oats can be recommended. A computerised literature review of multiple databases was carried out, identifying 17 primary studies, 6 of which met the criteria for inclusion in this review. None of the six studies found any significant difference in the serology between the oats and control groups. Two studies, however, identified a significant difference (p<0.001; p = 0.039) in intraepithelial lymphocyte counts between the oats and control groups. Oats can be symptomatically tolerated by most patients with coeliac disease; however, the long-term effects of a diet containing oats remain unknown. Patients with coeliac disease wishing to consume a diet containing oats should therefore receive regular follow-up, including small bowel biopsy at a specialist clinic for life.

Cutaneous reactions to foods represent one of the most common presentations of food allergy in children. IgE-mediated (urticaria, angioedema, flushing, pruritus), cell-mediated (contact dermatitis, dermatitis herpetiformis), mixed IgE- and cell-mediated (atopic dermatitis), and nonimmune-mediated (irritant contact dermatitis, Frey's syndrome) reactions to foods have all been reported. It is important for the pediatrician to recognize the variety of skin reactions potentially related to food allergy and to consider timely referral to an allergy specialist for further evaluation and definitive diagnosis.