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The current knowledge on the associations between coeliac disease and different skin diseases is contradictory and the patient's perspective on the burden of these is lacking. This study aimed to investigate patient-reported frequency, severity and quality of life effects of skin disorders in coeliac disease patients compared to controls and moreover to study the impacts of gluten-free diet on these skin diseases. A study questionnaire designed for the purposes of this study and a validated Dermatology Life Quality Index (DLQI) questionnaire were posted to 600 adult members of the Finnish Coeliac Society and 1173 matched controls. Responses from 327 coeliac disease patients and 382 non-coeliac controls were compared. Coeliac disease patients were shown to be at no increased risk of atopic dermatitis, acne, rosacea, psoriasis, alopecia areata, vitiligo or chronic urticaria. The severity of these skin diseases did not differ between study groups, but the risk for at least moderate effects on quality of life caused by dermatological diseases was increased among those with coeliac disease. Positive response from gluten-free diet was most commonly experienced by coeliac disease patients with atopic dermatitis. Even though the risk for skin diseases was shown not to be increased among coeliac disease patients, there is still an increased burden related to experienced skin symptoms among these patients, which non-dermatologists treating coeliac disease patients should acknowledge. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Dermatitis herpetiformis (DH) is an inflammatory skin disorder often considered as an extra intestinal manifestation of celiac disease (CeD). Hallmarks of CeD and DH are auto‐antibodies to transglutaminase 2 (TG2) and transglutaminase 3 (TG3), respectively. DH patients have auto‐antibodies reactive with both transglutaminase enzymes. Here it is reported that in DH both gut plasma cells and serum auto‐antibodies are specific for either TG2 or TG3 with no TG2–TG3 cross reactivity. By generating monoclonal antibodies from TG3‐specific duodenal plasma cells of DH patients, three conformational epitope groups are defined. Both TG2‐specific and TG3‐specific gut plasma cells have few immunoglobulin (Ig) mutations, and the two transglutaminase‐reactive populations show distinct selection of certain heavy and light chain V‐genes. Mass spectrometry analysis of TG3‐specific serum IgA corroborates preferential usage of IGHV2‐5 in combination with IGKV4‐1. Collectively, these results demonstrate parallel induction of anti‐TG2 and anti‐TG3 auto‐antibody responses involving separate B‐cell populations in DH patients. [ABSTRACT FROM AUTHOR]

Background: Dermatitis herpetiformis is a rare autoimmune bullous dermatosis that predominantly affects Caucasians, adults or children, with a sex ratio of 1.8. Materials and Methods: The aim of this study was to document the clinical and epidemiological profiles of dermatitis herpetiformis and to detail its treatment in a hospital setting in order to increase our knowledge about this disease in our context and so to improve its management. We conducted a retrospective, cross-sectional study of the records of all patients seen in consultation or hospitalized at the dermatology department of Yalgado Ouédraogo University Hospital Centre, Ouagadougou, a public hospital in Burkina Faso, from January 2016 to December 2020. Results: We collected 14 cases (0.12%) of dermatitis herpetiformis among 11,456 patients seen. The mean age was 8 years (ranging from 4 to 27 years). The sex ratio was 1.33. The majority of the patients were schoolchildren living in rural areas (8 cases). The duration of the disease ranged from five days to one year (mean duration: 59.35 days). Eight patients had a history of digestive problems such as abdominal pain and diarrhea. Pruritis was the principal functional sign. In all patients, the lesions were polymorphous: disseminated vesicular bullae, papules, erosive, and excoriated lesions, sometimes forming clusters. Mucosal involvement was rare (3 cases). A gluten-free diet and dapsone 2 mg/kg/day were proposed to all patients and resulted in the improvement of the lesions. Conclusion: Our study confirmed that dermatitis herpetiformis is rare in our context. It is more frequent in young children and predominantly affects boys. It is intensely pruritic, and generalized polymorphous lesions were present in all our patients. Treatment is essentially based on a gluten-free diet and dapsone, which is a therapeutic test in the absence of supplementary investigations to establish a definitive diagnosis. [ABSTRACT FROM AUTHOR]

Dermatitis herpetiformis has been investigated in the past; however, only a limited number of studies have reported its incidence based on validated nationwide population-based registries. To address this gap, the aims of this study are to estimate the incidence of dermatitis herpetiformis in Sweden and to validate the National Patient Register (NPR) for diagnosis of dermatitis herpetiformis. A population-based open cohort study was conducted, including all patients diagnosed with dermatitis herpetiformis (International Classification of Diseases 10th revision; ICD-10 code L13.0) in Sweden from 2005 to 2018 (n=1,724), identified from the NPR. The diagnosis of dermatitis herpetiformis in the NPR was validated using medical records, histopathological and immunopathological data, yielding a positive predictive value (PPV) of 62.5%. The mean annual incidence of dermatitis herpetiformis was 0.93/100,000 (95% confidence interval 0.79–1.08), female to male ratio 1:1, and mean age at diagnosis 60.9 years. In conclusion, this large nationwide cohort study showed a low validity for diagnosis of dermatitis herpetiformis in the NPR, and the adjusted incidence rate of dermatitis herpetiformis in Sweden was estimated to be 0.93/100,000, which is lower than that in previous Swedish studies. [ABSTRACT FROM AUTHOR]

Background: An increased risk of kidney disease in patients with celiac disease has been reported, but the association has remained obscure. Only few studies have investigated the association between renal comorbidities and dermatitis herpetiformis, a cutaneous manifestation of celiac disease. Objectives: We investigated whether patients with different phenotypes of celiac disease are at higher risk of kidney diseases than age‐ and sex‐matched references. Methods: The diagnoses of glomerulonephritis, diabetic nephropathy, interstitial nephritis, and end‐stage renal disease obtained from the National Hospital Discharge Register between 1970 and 2015 were identified in celiac disease (Marsh III, n = 1072) and dermatitis herpetiformis (n = 368) patients diagnosed at Tampere University Hospital catchment region and in 4296 reference subjects. Using the Cox proportional hazards model, we compared the risk of kidney diseases between patients and references. The study protocol was approved by the Regional Ethics Committee of Tampere University Hospital (R16090). As the study was register based, no consent from patients was required. Results: Even after adjusting for type 1 diabetes, celiac disease was associated with an elevated risk of kidney disease (hazard ratio [HR] 1.85, 95% confidence interval [CI] 1.12–3.03), glomerulonephritis (HR 3.37, 95% CI 1.64–6.95), and IgA nephropathy (IgAN) (HR 18.98, 95% CI 2.29–157.63). No similarly elevated risk was found among dermatitis herpetiformis patients (HR 1.50, 95% CI 0.63–3.55; HR 2.21, 95% CI 0.77–6.38; and HR 5.87, 95% CI 0.53–64.79, respectively). Conclusion: Celiac disease patients were at increased risk of kidney diseases, notably IgAN. The risk was dependent on the celiac disease phenotype and was not seen in patients with dermatitis herpetiformis. Awareness of possible renal manifestations is recommended when treating celiac disease patients. [ABSTRACT FROM AUTHOR]

BACKGROUND: Dermatitis herpetiformis is a rare, autoimmune, pruritic disease that is infrequent in children and adolescents, considered a cutaneous manifestation of gluten intolerance. It is characterized by erythematous papules and grouped vesicles that can adopt a herpetiform distribution, symmetrically distributed on extensor surfaces of the upper and lower extremities, elbows, knees, scalp, neck and buttocks. CLINICAL CASE: An 18-year-old male patient who came to the consultation complaining of symmetrically arranged vesicles and papules, crusts and hyperpigmentation in the gluteal and inguinal region, elbows and neck; the biopsy confirmed the diagnosis of dermatitis herpetiformis. The patient was treated with dapsone with favorable evolution. CONCLUSIONS: We present a case of dermatitis herpetiformis in an adolescent, which is unusual in this age group. [ABSTRACT FROM AUTHOR]

Autoimmune bullous skin disorders are a group of disorders characterized by the formation of numerous blisters and erosions on the skin and/or the mucosal membrane, arising from autoantibodies against the intercellular adhesion molecules and the structural proteins. They can be classified into intraepithelial or subepithelial autoimmune bullous dermatoses based on the location of the targeted antigens. These dermatoses are extremely debilitating and fatal in certain cases, depending on the degree of cutaneous and mucosal involvement. Effective treatments should be implemented promptly. Glucocorticoids serve as the first-line approach due to their rapid onset of therapeutic effects and remission of the acute phase. Nonetheless, long-term applications may lead to major adverse effects that outweigh the benefits. Hence, other adjuvant therapies are mandatory to minimize the potential harm and ameliorate the quality of life. Herein, we summarize the current therapeutic strategies and introduce promising therapies for intractable autoimmune bullous diseases. [ABSTRACT FROM AUTHOR]

Aim: Autoimmune bullous diseases occur when antibodies against proteins such as desmosomes and hemidesmosomes cause separation in the skin. Detailed epidemiological studies examining these diseases are limited in our country. We aim to contribute to the demographic, clinical, and treatment data on bullous diseases from an important geographic region of our country. Materials and Methods: The data of 125 cases who were followed up with a diagnosis of bullous disease between 2006 and 2019 were evaluated retrospectively. Socio-demographic characteristics, diagnoses, age and duration of disease onset, clinical findings, accompanying systemic diseases, treatments given, and side effects related to treatment were recorded. Results: 57.6% of the patients (72 people) were diagnosed as pemphigus, 31.2% (39 people) bullous pemphigoid (BP), 8% (10 people) dermatitis herpetiformis (DH), 3.2% (4 people) linear Ig A dermatosis ( LAD). BP patients were found to be significantly older than the other patients (p = 0.002). While pemphigus, BP, and LAD were more common in women, 70% of DH patients were men. Malignancy was detected in 2.8% of pemphigus patients and 5.1% of BP patients. Accompanying autoimmune disease was found in 9.7% of pemphigus patients, 17.9% of BP patients, and 20% of DH patients. The only systemic steroid was used in 20.8% of pemphigus patients, and azathioprine as a conventional agent was used in 76.4% with systemic steroid. Rituximab was started in patients who did not respond to therapy. The systemic steroid was used in 35.9% of BP patients, and azathioprine with systemic steroid was used in 43.6% of BP patients. Doxycycline was added to the treatment in 41% of the patients. Rituximab was used in 5 (12.8%) patients. Conclusion: Since our study is the first data obtained on bullous diseases from the southeastern Anatolia region, it is useful for the literature in terms of providing a basis for future studies. [ABSTRACT FROM AUTHOR]

Dermatitis herpetiformis (DH) is the skin manifestation of celiac disease, presenting with a blistering rash typically on the knees, elbows, buttocks and scalp. In both DH and celiac disease, exposure to dietary gluten triggers a cascade of events resulting in the production of autoantibodies against the transglutaminase (TG) enzyme, mainly TG2 but often also TG3. The latter is considered to be the primary autoantigen in DH. The dynamics of the development of the TG2-targeted autoimmune response have been studied in depth in celiac disease, but the immunological process underlying DH pathophysiology is incompletely understood. Part of this process is the occurrence of granular deposits of IgA and TG3 in the perilesional skin. While this serves as the primary diagnostic finding in DH, the role of these immunocomplexes in the pathogenesis is unknown. Intriguingly, even though gluten-intolerance likely develops initially in a similar manner in both DH and celiac disease, after the onset of the disease, its manifestations differ widely. [ABSTRACT FROM AUTHOR]

Although relatively uncommon, autoimmune bullous diseases carry the risk of increased mortality and can significantly impact quality of life. This group of diseases is broad and encompasses subepidermal conditions such as bullous pemphigoid, cicatricial pemphigoid, epidermolysis bullosa acquisita, dermatitis herpetiformis, and linear IgA bullous dermatosis, as well as intraepidermal conditions such as pemphigus and its variants. The pathophysiology of each condition is incompletely understood but broadly involves the formation of autoantibodies targeting skin adhesion proteins, a process that relies on a complex interplay between a dysregulated immune system, genetic predisposition, and environmental factors. We review the impact of nutrition on pathogenesis, clinical course, and treatment of various autoimmune bullous diseases. [ABSTRACT FROM AUTHOR]

Dermatitis herpetiformis is a rare chronic, autoimmune bullous disease linked to gluten sensitivity with intense pruritus and characteristic skin eruptions. Etiopathogenesis is complex and not fully understood. It is currently considered to be a specific cutaneous manifestation of celiac disease. Genetic, environmental and immunological factors influence both conditions. Exposure to gluten is the starting point of an inflammatory cascade leading to the formation of circulating IgA antibodies against tissue transglutaminase and skin immune IgA deposition followed by skin lesions. Binding of the immune complex deposits of IgA transglutaminases and epidermal antibodies with enzymes in the papillary dermis stimulates complement activation, neutrophil influx, proinflammatory cytokine release and overproduction of matrix metalloproteinases. We have collected current knowledge of the pathogenesis of dermatitis herpetiformis. [ABSTRACT FROM AUTHOR]

Dermatitis herpetiformis is a blistering autoimmune skin disease, and a cutaneous manifestation of coeliac disease. The burden of coeliac disease is increased especially in females, but studies concerning sex differences in patients with long-term treated dermatitis herpetiformis are scarce. This questionnaire study compared adherence to a gluten-free diet, clinical symptoms and well-being between females and males in a cohort of 237 long-term treated (median 24 years) patients with dermatitis herpetiformis. Females had better adherence to a gluten-free diet (p = 0.022) and they used dapsone significantly less often at the time of the study than did males (4% vs 13%, p = 0.017). The occurrence of skin symptoms was equal in both sexes, but dermatological quality of life was lower in females (p = 0.024), and gastrointestinal symptoms were more severe among females with dermatitis herpetiformis than among males (p = 0.027). In conclusion, long-term treated female patients with dermatitis herpetiformis have better adherence to a gluten-free diet, but they also experience more severe clinical symptoms compared with males. [ABSTRACT FROM AUTHOR]

Coeliac disease (CD) is a systemic disease of the unwarranted immune reaction to gluten and is associated with a 10% increased risk of cardiovascular disease. Here we present a patient with recurrent myocardial ischemia and intractable itching who was eventually diagnosed with CD. A 53-year-old man presented to the allergy department due to intractable itching that was resistant to antihistamine therapy. In addition, despite successful percutaneous intervention with stent implantation to the right coronary artery, there was an ST segment elevation with myocardial infarction (MI) in the lower wall that had occurred three times. After dermatitis herpetiformis was reported as a result of the biopsy performed from the lesions, duodenal biopsy confirmed the diagnosis of CD. Diagnosis of CD with atypical presentation can be difficult. Cardiovascular risk is increased in patients with celiac disease compared to the normal population. Itching is an important symptom that needs to be evaluated in detail, even without the typical gastrointestinal manifestations of CD. [ABSTRACT FROM AUTHOR]

Dermatitis herpetiformis is a cutaneous manifestation of coeliac disease treated with a gluten-free diet. However, the itching and blistering rash alleviates slowly after gluten withdrawal and occasionally persists despite a long-term gluten-free diet. This study investigated the prevalence and factors associated with prolonged (i.e. > 2 years) and ongoing skin symptoms in 237 patients with dermatitis herpetiformis. Data were gathered from medical records and via questionnaires. Among patients with dermatitis herpetiformis, 38% had prolonged symptoms after diagnosis, and 14% had ongoing skin symptoms at follow-up (median duration of gluten-free diet 24 years). A severe rash at diagnosis was associated with both prolonged and ongoing cutaneous symptoms. In addition, patients with dermatitis herpetiformis with ongoing skin symptoms at follow-up had been on the dietary treatment for a shorter time (median duration 16 vs 25 years) and were less often on a strict diet (53% vs 78%) compared with patients with dermatitis herpetiformis without ongoing skin symptoms. [ABSTRACT FROM AUTHOR]

Stiff person syndrome (SPS) is a rare disease affecting the central nervous system which can be autoimmune, paraneoplastic or idiopathic in origin. Its typical classic presentation is characterized by progressive stiffness of the trunk and limbs, associated with spasms. The diagnosis is supported by the existence of continuous and spontaneous muscle activity on electroneuromyogram detection, the presence of serum anti-GAD antibodies, and a response to benzodiazepines. We report the case of a 46-year-old patient with a classic form of autoimmune stiff person syndrome associated with dermatitis herpetiformis. [ABSTRACT FROM AUTHOR]

Immunobullous diseases are blistering cutaneous disorders that are caused by pathogenic antibodies binding to protein targets within the skin. There are a range of immunobullous disorders with characteristic morphology that relates to the structural properties of the target protein. In this article we will describe the pathogenesis, clinical features and treatment of the most common immunobullous disorders. [ABSTRACT FROM AUTHOR]

Granular deposits of IgA represent the specific cutaneous marker of dermatitis herpetiformis. The prevalence of IgA deposits in the skin of patients with coeliac disease without dermatitis herpetiformis remains unknown. In this prospective case-control study, skin biopsies from newly diagnosed coeliac patients without dermatitis herpetiformis were analysed by direct immunofluorescence. Controls included healthy volunteers and patients with both bowel symptoms and skin eruptions unrelated to coeliac disease. Clinical data and serum level of anti-tissue transglutaminase and anti-epidermal transglutaminase IgA antibodies were collected from patients and controls. Granular deposits of IgA or IgA1 in the skin were found in 29 out of 45 patients with coeliac disease (64.4%), and in none of the included controls (specificity 100%; sensitivity 64.4%). Positive direct immunofluorescence correlated significantly with an increased serum level of anti-epidermal transglutaminase IgA antibodies (p < 0.005). This study shows that granular deposits of IgA represent a low sensitive, but highly specific, cutaneous marker of coeliac disease independent of dermatitis herpetiformis. [ABSTRACT FROM AUTHOR]

Chronic plaque psoriasis is often associated with autoimmune bullous diseases. Dermatitis herpetiformis (DH) is a rare immunobullous disease that has been linked to celiac disease (CD). To our knowledge, the coexistence of psoriasis and DH is uncommon, and has only been described in anecdotal reports. We report a case of chronic plaque psoriasis complicated by DH in a 60-year-old patient with no known history of CD or associated symptoms. In our patient, DH presented atypically as multiple vesicles along the edges of psoriatic plaques located on the back and hips, and as vesiculobullous eruptions on the fingers. The patient was successfully treated with a combination of dapsone and a gluten-free diet for DH, and secukinumab for psoriasis. This case highlights the importance of screening for CD in patients with psoriasis, as well as other concomitant autoimmune diseases. A gluten-free diet should be trialled in psoriatic patients with positive CD serology. [ABSTRACT FROM AUTHOR]

INTRODUCTION: We assessed whether celiac disease-associated mortality is increased in Finland among patients diagnosed in the 21st century, given recent improvements in diagnostic and treatment facilities. METHODS: Biopsy-proven patients with celiac disease (Marsh III) and dermatitis herpetiformis aged 20-79 years (median 50 years) diagnosed 2005-2014 (n = 12,803) were identified from the national dietary grant registry. Dates and causes of death were obtained from Statistics Finland. Overall mortality and causes of death were compared with reference individuals (n = 38,384) matched for age, sex, and area of residence (at the time of celiac disease diagnosis) selected from the Population Information System. RESULTS: During a mean follow-up of 7.7 years (SD ±3.0years), 884(6.9%)and 2,613 (6.8%) deaths occurred among the celiac cohort and reference group, respectively. Overall mortality (hazard ratio [HR] 1.01, 95% confidence intervals [CIs] 0.94-1.09), mortality from all malignancies (HR 1.11, 95% CI 0.96-1.27), gastrointestinal tract malignancies (HR 1.21, 95% CI 0.56-1.71), or cardiovascular diseases (HR 0.91,95% CI 0.77-1.07) were not increased among patients with celiac disease. Overall, mortality from lymphoproliferative diseases (HR 2.36,95% CI 1.65-3.39) and non malignant digestive diseases (HR 2.19, 95% CI 1.40-3.43) was increased, but HRs decreased after the exclusion of the first 2 years of follow-up (HR 1.71,95% CI 1.10-2.66 and HR 1.75,95% CI 1.01-3.05, respectively). DISCUSSION: The overall mortality in adult celiac disease diagnosed 2005-2014 was not increased. Mortality from lymphoproliferative diseases was increased but lower than previously reported. [ABSTRACT FROM AUTHOR]

Dermatitis herpetiformis (DH) is an autoimmune skin disease that causes itchy, blistering rash, typically on the elbows, knees and buttocks. DH and coeliac disease share the same genetic background, glutendependent enteropathy and antibody response against tissue transglutaminase. DH is currently considered a cutaneous manifestation of coeliac disease, and the prevailing hypothesis is that DH develops as a late manifestation of subclinical coeliac disease. The incidence of DH is decreasing contemporarily with the increasing incidence of coeliac disease. The IgA immune response in DH skin is directed against epidermal transglutaminase, while the autoantigen in the gut is tissue transglutaminase. Granular IgA deposition in the papillary dermis is pathognomonic for DH, and is a finding used to confirm the diagnosis. The treatment of choice for DH is a life-long gluten-free diet, which resolves the rash and enteropathy, increases quality of life, and offers a good long-term prognosis. [ABSTRACT FROM AUTHOR]

Introduction. Dermatitis herpetiformis is a chronic autoimmune bul-lous disease related to gluten intolerance with a polymorphic form of lesions that imitates other dermatoses and causes important diagnostics difficulties. Objective. To report a case of a very rarely diagnosed haemorrhagic form of Duhring disease, which developed in a 72-year-old man. Case report. This article presents a description of a patient with lesions located on smooth body skin and scalp, which lasted for several months and had the character of erythematopapular eruptions with a haemor-rhagic component and secondary erosions and scabs. The skin lesions had an annular structure and the distribution was symmetrical with the greatest intensity at the elbows, knees, buttocks and thighs. Histopa-thological examination, direct immunofluorescence, indirect immunofluorescence and the immunoblot method allowed diagnosing Duhring disease. Incorporated pharmacological treatment with dapsone and a gluten-free diet resulted in a significant clinical improvement. Conclusions. The presented case draws attention to atypical clinical presentation and the appearance time of the first symptoms of dermatitis herpetiformis. [ABSTRACT FROM AUTHOR]

This mini-review presents an update on the direct immunofluorescence (DIF) for diagnosing dermatitis herpetiformis. The DIF of uninvolved, perilesional skin is a crucial laboratory procedure in diagnosing dermatitis herpetiformis (DH). IgA deposits at the dermal-epidermal junction (DEJ) of perilesional skin with DIF can also be found in coeliac patients with inflammatory skin diseases different from DH. In certain patients presenting with the rash resembling DH, the deposition of exclusively C3 at DEJ can be found. The term "granular C3 dermatosis" was proposed to name such a rash. Recent data on DH suggest that perhaps the very concept of DH that we are universally accepting now is misleading and should be revised. [ABSTRACT FROM AUTHOR]

Dermatitis herpetiformis is a rare, chronic, autoimmune blistering disease with hypersensitivity to gluten with the presence of intense pruritus. Symptoms of the disease are symmetrical, polymorphic skin lesions in a typical location. It is thought that dermatitis herpetiformis is a specific skin manifestation of celiac disease, with frequent asymptomatic gluten enteropathy. Both diseases are characterized by the same tissue compatibility antigens, the presence of circulating IgA antibodies against endomysium and transglutaminases, and clinical remission on a glutenfree diet. Non-adherence to this diet predisposes to the development of some tumours, autoimmune and thyroid gland diseases. The diagnosis of dermatitis herpetiformis is based on the findings of the characteristic IgA deposits in the skin papilla in the direct immunofluorescence test. Specific serum antibodies are used as an aid in the diagnosis and for monitoring the compliance with gluten free diet. [ABSTRACT FROM AUTHOR]

Objectives: Dermatitis herpetiformis (DH) is a cutaneous manifestation of coeliac disease. Bone fracture risk is increased in coeliac disease, but little knowledge exists about bone complications in DH. This study aimed to evaluate the risk of hip and other hospital-treated fractures in DH and coeliac disease in a high prevalence area with good adherence to a gluten-free diet. Materials and methods: Hip, proximal humerus, wrist and ankle fractures in 368 treated DH and 1076 coeliac disease patients between 1970 and 2015 were reviewed from the National Hospital Discharge Register. Hip fracture incidence rates for DH and coeliac disease patients were compared to those for the general population. The overall fracture risk for DH was compared to coeliac disease. Results: The hip fracture incidence rates for DH and coeliac disease patients did not differ from the general population. In females aged 80–89, the hip fracture incidence was higher in DH than in coeliac disease, but the risk for any hospital-treated fracture was lower in DH compared to coeliac disease (adjusted HR 0.620, 95% CI 0.429–0.949). The DH and coeliac disease patients with hospital-treated fractures were diagnosed at an older age, but the degree of small bowel mucosal damage did not significantly differ between patients with and without fractures. Conclusion: The incidence of hip fracture is not increased in treated DH or coeliac disease in an area with high awareness and dietary compliance rates. However, patients with DH seem to have a lower risk for fractures overall compared to coeliac disease. [ABSTRACT FROM AUTHOR]

Background and purpose: Magnitude of gluten‐specific T‐cell responses in coeliac disease (CD) might be dependent on HLA‐DQ2 gene dose. We aimed to investigate the effects of HLA‐DQB1*02 allele dose on clinical outcomes. Methods: We reviewed the charts of all coeliac patients attending to three Hungarian university clinics after 1997 and included those patients, who (a) were diagnosed with CD, (b) underwent high‐resolution HLA typing and (c) were ≥18 years at the time of data collection. HLA typing was performed to determine DQB1*02 allele dose. Patients were divided into risk groups by DQB1*02 allele dose, as follows: high‐, intermediate‐ and low‐risk groups corresponded to a double, single and zero doses, respectively. We used ANOVA and Pearson's chi‐squared test to explore association between HLA risk and clinical variables. Results: A total of 727 coeliac patients attended the clinics but only 105 (14.4%) patients were eligible for inclusion. High, intermediate and low HLA risk patients comprised 35.3%, 52.3% and 12.3% of the study population, respectively. Double dose of HLA‐DQB1*02 was more frequent in patient with high tTGA level (>10 times the upper limit of normal; p = 0.045). Gene dose was not associated with younger age at diagnosis (p = 0.549), gender (p = 0.739), more severe diagnostic histology (p = 0.318), more frequent classical presentation (p = 0.846), anaemia (p = 0.611), metabolic bone disease (p = 0.374), dermatitis herpetiformis (p = 0.381) and autoimmune diseases (p = 0.837). Conclusions: Our study shows a significant gene dose effect in terms of tTGA level at diagnosis, but no significant association between HLA‐DQB1*02 allele dose and the clinical outcomes in CD. [ABSTRACT FROM AUTHOR]

A 45-year-old man suffering from dermal blistering disease with proteinuria and hematuria underwent renal biopsy. The renal biopsy specimen suggested proliferative glomerulonephritis with monoclonal IgG deposits under routine light, immunofluorescence and electron microscopy. The staining for IgG subclasses (IgG1 and IgG2) and κ/λ light chain indicated secondary immune complex type MPGN type 3. The patient had been diagnosed as having dermatitis herpetiformis (DH), a phenotype of gluten hypersensitivity prior to the appearance of the renal abnormality. Although common autoantibodies might be related to the pathogenesis of disorders in the skin and kidney, DH is mainly driven by IgA autoantibody, while MPGN is induced by IgG immune complexes. IgA was not observed in the glomeruli by immunofluorescence. Neither the examination for DH specific autoantibodies nor HLA-DQB1 genotype supported the diagnosis of DH. Reassessment of the skin biopsy record revealed that the blister was localized in the epidermis, suggesting pemphigus herpetiformis by IgG class anti-epidermal autoantibody, which also affected the renal disorder. [ABSTRACT FROM AUTHOR]

Objectives. Autoimmune diseases (AD) may be associated with coeliac disease (CD), but specific risk factors have been poorly investigated. The aim of this study was to assess the spectrum of AD and its specific risk factors associated in a series of adult coeliac patients. Materials and Methods. We performed a single-center case-control study including adult newly diagnosed CD patients. To evaluate the risk factors of the association between AD and CD, 341 coeliac patients included were categorized on the basis of AD presence: 91 cases with at least one AD and 250 controls without AD were compared for clinical, serological, and histological features. Eighty-seven cases were age-gender-matched with 87 controls. Results. Among 341 CD patients, 26.6% of CD patients had at least one AD. Endocrine and dermatological diseases were the most prevalent AD encountered: autoimmune thyroiditis was present in 48.4% of cases, psoriasis in 17.6%, and type I diabetes and dermatitis herpetiformis in 11%, respectively. At logistic regression, factors associated with AD were a positive 1st-degree family history of AD (OR 3.7, 95% CI 1.93–7), a body mass index ≥ 25 kg/m2 at CD diagnosis (OR 2.95%, CI 1.1–3.8), and long standing presentation signs/symptoms before CD diagnosis (>10 years) (OR 2.1, 95% CI 1.1–3.7). Analysis on age-gender-matched patients confirmed these results. Conclusions. CD patients with family history of AD, overweight at CD diagnosis, and a delay of CD diagnosis had an increased risk of having another AD. The benefit of CD screening in these specific subsets of patients with AD awaits further investigation. [ABSTRACT FROM AUTHOR]

Abstract: Background: Pemphigus herpetiformis (PH) is a rare subtype of pemphigus combining clinical features of dermatitis herpetiformis and the immunopathologic characteristics of pemphigus. We aimed to analyze the epidemiological, clinical, and immunological presentation and management of the disease in a cohort of south Tunisian patients with a long‐term follow‐up. Methods: We included all patients with confirmed PH followed from January 1987 to December 2015 in the dermatology department. Results: We included 24 south Tunisian female patients with a mean age of 36 years (20–63), mainly from rural origin (84%). All patients had exclusive skin lesions. Clinical features combined erythematous, vesicular, or bullous lesions, with circinate borders mainly on the trunk and limbs associated with severe pruritus (91%). Peripheral hypereosinophilia was noted in 31% of cases. Histological examination demonstrated no intraepithelial (50%), suprabasal (35%), or subcorneal cleavage (15%). Eosinophilic spongiosis was seen in 42% of cases. On direct immunofluorescence, there was an intercellular staining in 96% of cases composed mainly of IgG and C3 (79%). Indirect immunofluorescence was positive in 71% of cases. Reactivity was mainly directed against Dsg1. Dapsone alone was inefficient. All patients received systemic corticosteroids. Most patients (64%) relapsed. Iatrogenic complications and cutaneous infections were noted in 40% and 16% of cases, respectively. Conclusion: PH is characterized by the occurrence of exclusive skin lesions in young women with a good response to corticosteroids but not to Dapsone. Reactivity mainly against Dsg1 suggests that PH in south Tunisia is a particular variant of nonendemic pemphigus foliaceus. [ABSTRACT FROM AUTHOR]

Abstract: 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. [ABSTRACT FROM AUTHOR]

Abstract: Background: Atopic dermatitis (AD) is characterized by skin barrier dysfunction. Notably, a high number of nano‐scale protrusions on the surface of corneocytes, which can be expressed by the Dermal Texture Index (DTI), were recently associated with paediatric AD, loss‐of‐function mutations in filaggrin gene (FLG) and reduced levels of natural moisturizing factors (NMF). No study has so far examined the association between these parameters and monomeric filaggrin levels in adults. Objective: To determine DTI, monomeric filaggrin and NMF in healthy controls and a group of patients with controlled dermatitis. Methods: A total of 67 adults (20 healthy controls and 47 dermatitis patients) were included. In the patient population, a personal history of AD was diagnosed by the U.K. Working Party's Diagnostic Criteria. All participants were tested for FLG mutations (R501X, 2282del4, R2447X). Transepidermal water loss, monomeric filaggrin, DTI and NMF were measured. Results: In the patient population, 78.7% (37/47) had a history of AD and 59.5% (28/47) had FLG mutations. Patients had significantly higher levels of DTI and significantly lower levels of monomeric filaggrin and NMF compared to the 20 healthy controls. Among patients, reduced level of monomeric filaggrin and NMF correlated with the presence of FLG mutations and clinical phenotypes such as xerosis, palmar hyperlinearity and AD. Among healthy controls, DTI was significantly higher in the oldest age group compared to the two younger age groups. Conclusion: A significant difference in DTI, monomeric filaggrin and NMF levels was found when comparing dermatitis patients with healthy controls. These findings suggest that even mild dermatitis or non‐visible inflammation has a significant and negative effect on the skin barrier as inflammation is known to reduce filaggrin levels. DTI was significantly increased in aged individuals in the healthy control group, suggesting a gradual change in corneocyte morphology with age. [ABSTRACT FROM AUTHOR]

Dermatitis herpetiformis (DH) is an autoimmune blistering disease of the skin. It is a result of hypersensitivity to dietary gluten. Diagnosis of DH can be challenging in a low prevalence, resource-limited population. We present the case of an African woman who presented with clinical features of DH. [ABSTRACT FROM AUTHOR]

Abstract: Gluten‐related disorders are a complex group of diseases that involve the activation of the immune system triggered by the ingestion of gluten. Among these, celiac disease, with a prevalence of 1 %, is the most investigated, but recently, a new pathology, named nonceliac gluten sensitivity, was reported with a general prevalence of 7 %. Finally, there other less‐prevalent gluten‐related diseases such as wheat allergy, gluten ataxia, and dermatitis herpetiformis (with an overall prevalence of less than 0.1 %). As mentioned, the common molecular trigger is gluten, a complex mixture of storage proteins present in wheat, barley, and a variety of oats that are not fully degraded by humans. The most‐studied protein related to disease is gliadin, present in wheat, which possesses in its sequence many pathological fragments. Despite a lot of effort to treat these disorders, the only effective method is a long‐life gluten‐free diet. This Review summarizes the actual knowledge of gluten‐related disorders from a translational chemistry point of view. We discuss what is currently known from the literature about the interaction of gluten with the gut and the critical host responses it evokes and, finally, connect them to our current and novel molecular understanding of the supramolecular organization of gliadin and the 33‐mer gliadin peptide fragment under physiological conditions. [ABSTRACT FROM AUTHOR]

Coeliac disease and dermatitis herpetiformis (DH) are characterized by autoantibodies targeting transglutaminase (TG)2 and TG3, respectively. Previous studies show that TG2 antibodies are produced in the gut and can be assessed in organ culture of small-intestinal biopsies from patients with coeliac disease. Thus far, no studies have investigated TG3 antibodies in organ culture of biopsies from patients with DH, or exploited the method in DH. The aim of this study was to investigate TG3 and TG2 antibody responses in serum and small-intestinal biopsies from patients with DH with active disease, and from those in remission. The majority of patients with DH were negative for both serum and organ culture medium TG2-targeting antibodies. Surprisingly, patientswithactive DHsecretedTG3 antibodies into the culture medium despite seronegativity. In patients secreting high levels of TG3 antibodies into the culture medium, we also detected TG3-antibody-positive cells in the small-intestinal mucosa. These findings suggest that TG3 antibodies can be investigated in the organ culture system and that their secretion occurs in the small intestine, especially in active DH. [ABSTRACT FROM AUTHOR]

Dermatitis herpetiformis (DH) is an extra-intestinal manifestation of coeliac disease. The highest currently reported prevalence of DH is in Finland, but knowledge of diagnostic delay is limited. This study investigated the duration of rash prior to diagnosis in 446 patients with DH, analysing the results in 3 periods of 15 years. The diagnosis was considered delayed when the duration of rash before diagnosis was 2 years or longer. Factors associated with delayed diagnosis were analysed. Within the 45 years, the median duration of rash before diagnosis decreased significantly, from 12.0 to 8.0 months (p = 0.002) and the occurrence of a delayed diagnosis decreased from 47% to 25% (p = 0.002). Female sex, the presence of villous atrophy, and a diagnosis of DH before the year 2000 were significantly associated with delayed diagnosis. In conclusion, the present study showed that one-quarter of patients currently have a diagnostic delay of 2 years or more, which is far from ideal. [ABSTRACT FROM AUTHOR]

Dermatitis herpetiformis or Duhring-Brocq disease is a chronic, autoimmune, pleomorphic disease, characterized by lesions on extension surfaces, accompanied by intense pruritus, and is usually associated with celiac disease, gluten sensitivity, gluten sensitivity ataxia and some forms of IgA neuropathy. Two cases of dermatitis herpetiformis are presented in female patients and we make a brief review of the literature on the treatment of this pathology. [ABSTRACT FROM AUTHOR]

Background The frequency of coeliac disease (CD) has been on the rise over the past decades, especially in Western Europe, but current trends are unclear. Aim To research the recent temporal changes in the incidence of adult, biopsy-verified coeliac disease and dermatitis herpetiformis (DH) in Finland, a country with a high frequency of coeliac disease. Methods All coeliac disease and DH cases diagnosed at age 20-79 years during 2005-2014 were retrieved from a nationwide database documenting all applicants for monthly compensation to cover the extra cost of maintaining a gluten-free diet. This benefit is granted on the basis of histology, not socioeconomic status. Temporal trends in the annual incidences were estimated using Poisson regression analyses. Results The total incidence of coeliac disease decreased from 33/100 000 during the years 2005-2006 to 29/100 000 during 2013-2014. The mean annual incidence of coeliac disease was nearly twice as high among women as among men, 42 vs 22 per 100 000, respectively. For middle- and old-aged women, the average rate of decrease in incidence was 4.8% (95% CI 3.9-5.7) per year and for men 3.0% (1.8-4.1) ( P for linear trend <.001, for both). Similarly, the annual incidence of DH declined. For young adults, the rate of change remained low and nonsignificant throughout the period 2005-2014. Conclusions Although the awareness of coeliac disease has increased during the past decades, the incidence of biopsy-verified diagnoses is not increasing, which suggests that exposure to yet unidentified triggering factors for coeliac disease has plateaued among the Finnish adult population. [ABSTRACT FROM AUTHOR]

A family of eleven proteins comprises the Janus kinases (JAK) and signal transducers and activators of transcription (STAT) signaling pathway, which enables transduction of signal from cytokine receptor to the nucleus and activation of transcription of target genes. Irregular functioning of the cascade may contribute to pathogenesis of autoimmune diseases; however, there are no reports concerning autoimmune bullous diseases yet to be published. The aim of this study was to evaluate the expression of proteins constituting the JAK/STAT signaling pathway in skin lesions and perilesional area in dermatitis herpetiformis (DH) and bullous pemphigoid (BP), as well as in the control group. Skin biopsies were collected from 21 DH patients, from 20 BP patients, and from 10 healthy volunteers. The localization and expression of selected STAT and JAK proteins were examined by immunohistochemistry and immunoblotting. We found significantly higher expression of JAK/STAT proteins in skin lesions in patients with BP and DH, in comparison to perilesional skin and the control group, which may be related to proinflammatory cytokine network and induction of inflammatory infiltrate in tissues. Our findings suggest that differences in the JAK and STAT expression may be related to distinct cytokines activating them and mediating neutrophilic and/or eosinophilic infiltrate. [ABSTRACT FROM AUTHOR]

The vesiculobullous variant of erythema elevatum diutinum ( EED) is a very rare variant of EED. We describe a 16-year-old boy who presented with symmetrical nodular lesions accompanied by vesicles on the dorsa of his hands. Biopsy findings were consistent with EED. The histopathological presence of IgA and neutrophils in the vesicles indicates that the joint action of both is responsible for formation of these subepidermic vesicles. We hypothesize that absence of human leucocyte antigen related to dermatitis herpetiformis ( DH) in our patient might have influenced the location and distribution of the lesions, so that they were not typical of DH. We report the second case of the vesiculobullous variant of EED with IgA deposits in the dermoepidermal membrane. To our knowledge, there are only 14 previously reported cases of the vesiculobullous variant of EED. [ABSTRACT FROM AUTHOR]

Inflammation-induced heat shock proteins (HSPs) and hypovitaminosis D have been reported to impact immune responses and to be associated with autoimmune diseases including celiac disease (CD), a gluten-sensitive enteropathy mediated by autoantibodies against tissue transglutaminase (TG2). Recently, we provided evidence for a role of autoantibodies to HSPs in patients with dermatitis herpetiformis (DH), an inflammatory skin disease induced by underlying latent CD. In this study, we aimed at investigating the humoral autoimmune response to HSPs and vitamin D status in CD patients ( n = 15) presenting without the cutaneous disease manifestation. In comparison with healthy controls ( n = 15), circulating autoantibodies against HSP40, HSP60, and HSP90 were increased in these patients, and these autoantibodies, including anti-HSP70, correlated with serum anti-TG2 autoantibodies. Deficient and insufficient vitamin D serum levels were found in 8 out of 15 CD patients and 11 out of 15 healthy controls, and the vitamin D status showed no relationship with levels of circulating anti-TG2 or anti-HSP autoantibodies in the patients. Our results preliminarily suggest a pronounced anti-HSP autoimmune response in CD that is linked to pathophysiological processes in the gut independently of both a potential concomitant disease-related extra-intestinal inflammatory manifestation in the skin and the vitamin D status. Future studies on larger groups of patients are needed to confirm the present data and to further clarify whether autoimmunity towards HSPs is primarily related to underlying CD or skin inflammation in DH patients. [ABSTRACT FROM AUTHOR]

Background Scalp is a common location of autoimmune bullous diseases. Trichoscopy is a noninvasive method for diagnosing hair and scalp diseases. Data on trichoscopy in autoimmune bullous diseases are limited to the studies on pemphigus including a small number of patients. Trichoscopic characteristics of bullous pemphigoid and dermatitis herpetiformis were not reported to date. The aim of the study was to determine the value of trichoscopy in the differential diagnosis of pemphigus vulgaris, pemphigus foliaceus, bullous pemphigoid, and dermatitis herpetiformis. Methods Trichoscopy was used to evaluate scalp lesions in 68 patients (26 with pemphigus vulgaris, 17 with pemphigus foliaceus, 17 with bullous pemphigoid, and 8 with dermatitis herpetiformis). The working magnification was 20-fold and 70-fold. Results The most frequent trichoscopic feature of autoimmune bullous diseases was extravasations. They occurred in 76.9% of patients with pemphigus vulgaris, 70.6% of patients with pemphigus foliaceus, 76.5% of patients with bullous pemphigoid, and 100% of patients with dermatitis herpetiformis. Yellow hemorrhagic crusts occurred in, respectively, 73.1%, 70.6%, 64.7%, and 35.5% of the cases. Yellow diffuse scaling and tubular scaling occurred more frequently in pemphigus foliaceus (52.9% and 41.2%, respectively). Clustered dotted vessels were characteristic for dermatitis herpetiformis (5/8, 62.5%). Dotted vessels with whitish halo were a hallmark of pemphigus vulgaris. A trichoscopic algorithm for the differential diagnosis of autoimmune bullous diseases was developed. Conclusions Autoimmune bullous diseases present characteristic trichoscopic patterns. Trichoscopy can be regarded as a rapid in-office preliminary diagnostic method in the differential diagnosis of these diseases. [ABSTRACT FROM AUTHOR]

Background: Data collected in EHRs have been widely used to identifying specific conditions; however there is still a need for methods to define comorbidities and sources to identify comorbidities burden. We propose an approach to assess comorbidities burden for a specific disease using the literature and EHR data sources in the case of autoimmune diseases in celiac disease (CD).Methods: We generated a restricted set of comorbidities using the literature (via the MeSH® co-occurrence file). We extracted the 15 most co-occurring autoimmune diseases of the CD. We used mappings of the comorbidities to EHR terminologies: ICD-10 (billing codes), ATC (drugs) and UMLS (clinical reports). Finally, we extracted the concepts from the different data sources. We evaluated our approach using the correlation between prevalence estimates in our cohort and co-occurrence ranking in the literature.Results: We retrieved the comorbidities for 741 patients with CD. 18.1% of patients had at least one of the 15 studied autoimmune disorders. Overall, 79.3% of the mapped concepts were detected only in text, 5.3% only in ICD codes and/or drugs prescriptions, and 15.4% could be found in both sources. Prevalence in our cohort were correlated with literature (Spearman's coefficient 0.789, p = 0.0005). The three most prevalent comorbidities were thyroiditis 12.6% (95% CI 10.1-14.9), type 1 diabetes 2.3% (95% CI 1.2-3.4) and dermatitis herpetiformis 2.0% (95% CI 1.0-3.0).Conclusion: We introduced a process that leveraged the MeSH terminology to identify relevant autoimmune comorbidities of the CD and several data sources from EHRs to phenotype a large population of CD patients. We achieved prevalence estimates comparable to the literature. [ABSTRACT FROM AUTHOR]

Background/Objectives Mucosal-associated invariant T ( MAIT) cells are a novel subset of innate-like T-cells that are enriched in mucosal tissues. Their presence in human skin has only recently been recognised. We describe the expression of skin-tropic molecules on human skin MAIT cells at steady state and investigate their contribution to various dermatoses with known T-cell involvement. Methods To examine the expression of skin-tropic molecules by MAIT cells at steady state, we performed a flow cytometric analysis of blood and skin samples from healthy donors. To investigate any potential wider contribution of MAIT cells to skin disease, we examined psoriasis, alopecia areata and dermatitis herpetiformis biopsies using immunofluorescent staining to identify the proportion of T-cells expressing MAIT cell surface markers. Results We found that MAIT cells constituted a small population of T-cells in normal human skin, similar to the percentage found in peripheral blood. Like other skin T-cells, skin MAIT cells expressed high levels of the skin-associated markers, cutaneous lymphocyte antigen and CD103. In psoriasis and alopecia areata the proportion of MAIT cells was similar to that found in normal skin, but in dermatitis herpetiformis it was significantly elevated. Conclusions The expression of skin-tropic molecules by skin MAIT cells is consistent with their resident status in normal human skin. Our results suggest that MAIT cells may play a role in the pathogenesis of dermatitis herpetiformis. [ABSTRACT FROM AUTHOR]

Background/aim: The distribution and the demographic, etiological, and clinical features of autoimmune bullous diseases (ABDs) vary according to geographic regions of the world. The limited number of reported studies in Turkey are mostly retrospective and generally include only pemphigus. The aim of our study was to evaluate the demographic data, clinical features, associated triggering factors, and comorbidities in patients newly diagnosed with ABDs. Materials and methods: In this descriptive and prospective study, the demographic data, inhabited regions, smoking and drinking habits, presence of comorbidities, and specific and general potential triggering factors of ABDs considered by the patients were examined. Furthermore, the distribution of lesions and autoimmune bullous skin disorder intensity scores were recorded. Results: Of the total 59 ABD patients, 26 (44.1%) were diagnosed with pemphigus, 25 (42.4%) were diagnosed with pemphigoid, and 8 (13.5%) were diagnosed with dermatitis herpetiformis. Although there were some differences between the subgroups of ABDs, the onset of the disease was associated with a triggering factor (stress, foods, physical trauma, chemical agents, ultraviolet rays, medications, infections, malignancies, and pregnancy, in a decreasing frequency) in 96.6% of the patients. Autoimmune diseases, neuropsychiatric disorders, diabetes mellitus, and hypertension were found to be statistically significantly higher in the pemphigoid group compared to the pemphigus and dermatitis herpetiformis groups. Conclusion: Evaluation of triggering factors and comorbidities before starting treatment may assist in controlling the disease more swiftly and reducing the side effects of treatment in the management of ABDs. However, to evaluate the frequency and significance of our findings in order to determine whether they are statistically significant or not, we suggest a multicenter prospective clinical trial, which would include control groups and a higher number of patients for each of the disease groups and clinical subtypes. [ABSTRACT FROM AUTHOR]

In the present study, it was evaluated whether east saline groundwater concentration solution (ESGWc) exerted a favorable inhibitory effect on 2,4-dinitrochlorobenzene (DNCB)-induced allergic/atopic-like dermatitis (AD). AD was induced and boosted by sensitization with DNCB via topical application on the dorsal back skins. Mice with DNCB-induced AD were bathed in 100-, 200- and 400-fold diluted ESGWc. After 6 weeks bathing, changes to body weight, clinical skin severity scores, scratching behavior, serum total immunoglobulin (Ig)E levels, submandibular lymph node and spleen weights, splenic cytokine levels, skin cytokine mRNA expressions, antioxidant defense systems and superoxide anion productions were recorded to determine the effects of bathing on the histopathology of dorsal back skin tissues. All DNCB-induced mice demonstrated that the induction of AD through IgE-mediated hypersensitivities, oxidative stresses, activation of MMP and apoptosis of keratinocytes resulted in no significant differences in body weight between the different groups at each time point following initial sensitization. However, markers of DNCB-induced AD were significantly inhibited (P<0.05) in a concentration-dependent manner following bathing in all concentrations of ESGWc. The results obtained in the present study suggest that bathing in ESGWc may have favorable protective effects against DNCB-induced AD due to favorable systemic and local immunomodulatory effects, active cytoprotective anti-apoptotic effects, inhibitory effects of matrix metalloproteinase activity, and anti-inflammatory and antioxidative effects. [ABSTRACT FROM AUTHOR]

Dermatitis herpetiformis (DH), bullous pemphigoid (BP), and pemphigus vulgaris (PV) are autoimmune bullous skin conditions with eosinophilic and neutrophilic infiltrations. While cytokines are crucial for the affinity and activation of different leukocyte cells in the inflammation and blister formation, there are no studies concerning a role of IL-36. The goal of the study was to analyze whether interleukin 36 is involved in pathogenesis of DH, BP, and PV. And the second aim of the study was the estimation of correlation between IL-36 and IL-17 and titers of specific antibodies in these diseases. Expression of IL-36 and IL-17 was detected in serum in all DH, BP, and PV samples. Serum levels of IL-36 and IL-17α were statistically higher in DH, BP, and PV groups as compared to the control group. IL-36α levels were statistically higher in DH patients, as compared to patients with PV and BP. Our results showed that IL-36 may be helpful in the diagnostic and monitoring of the activity of the disease. IL-36 may play a relevant role of enrolling eosinophils and neutrophils in DH, BP, and PV and finally provoke tissue injury. [ABSTRACT FROM AUTHOR]