Dermatitis Herpetiformis Research Articles

Perineal cutaneous basal cell carcinomas in patients with familial seronegative celiac disease: Report of three cases

Introduction: Celiac disease (CD) is an autoimmune enteropathy due to hypersensitivity to consumed gluten. Patients can present with gastrointestinal and systemic symptoms. CD often runs in families. Studies have reported multiple subtypes of CD including at least one seronegative variant. The cutaneous manifestations of CD remain to be delineated; some cases of seropositive CD are associated with dermatitis herpetiformis. We report. Cases: In this study, three patients with a longstanding history of personal and/or familial seronegative CD presented withwho developed basal cell carcinoma (BCC) of the perineum. We hope to inform clinical physicians about the possible relationship between CD and cutaneous malignancies. DiscussionResults: We report three adult patients with a diagnosed or suspected personal history of seronegative CD and a family history of CD, who presented with BCC of the perineum. Discussion: The mechanism by which CD causes BCC remains to be defined. Physicians should consider testing evaluating patients with skin cancer located in the perineum for CD.

Rare Complications of Celiac Disease: Clinicopathologic Features

Celiac disease (CD) is an immune mediated disorder characterised by intolerance to glutens in certain grains like whet, barley, and rye. The exposure to gliadin protein component in the susceptible individuals leads to an inflammatory reaction damaging small bowel mucosa with progressive disappearance of intestinal villi. The damaged intestinal mucosa leads to malabsorption. The usual symptoms of celiac disease include diarrhea, steatorrhea, weight loss, fatigue, and abdominal pain. Diagnosis is based on clinical features, duodenal biopsy, elevated levels of anti-gliadin antibodies and response to gluten free diet. Contrary to common belief, celiac disease is a protein systemic disease rather than merely a pure digestive alteration. Celiac disease is closely associated with genes that code HLA -II antigens mainly of DQ2 and DQ8 classes, production of disease specific antibodies (i.e., endomysial antibodies), multiorgan involvement, comorbidity with other autoimmune diseases (shared autoimmunity), familial aggregation, and immune system dysregulation. The clinical presentation of celiac disease can be variable. In mild form, patients can be almost asymptomatic whereas in the most severe form, the patients are at increased risk of life-threatening complications. Celiac disease has a well-known association with other autoimmune diseases such as autoimmune liver diseases (autoimmune hepatitis, primary biliary cirrhosis, primary sclerosing cholangitis), diabetes mellitus, autoimmune thyroid diseases, skin diseases such as dermatitis herpetiformis, rheumatoid arthritis, systemic lupus erythematosus, Sjogren’s syndrome, psoriasis, sarcoidosis, immune thrombocytopenic purpura, and pancreatitis. In addition, celiac disease may be associated with rare but potentially serious complications such as, collagenous sprue, ulcerative jejunoileitis, refractory celiac disease (RCD), enteropathy associated T-cell lymphoma, small bowel adenocarcinoma (SBA), hyposplenism, and cavitating mesenteric lymph node syndrome (CMLNS). The present article describes clinicopathologic features of these rare but serious complications of celiac disease.

Short Course Treatment with Dapsone in a 59-Year-Old Male with Dermatitis Herpetiformis: A Case Report

Dermatitis Herpetiformis is a chronic autoimmune blistering skin condition that is often associated with gluten-sensitive enteropathy. The primary lesion of DH is an erythematous papule, plaque, or, most commonly, a vesicle that can be pruritic.

Celiac Disease: a review

Celiac Disease (CD) is an immune-mediated enteropathy in response to the ingestion of gluten in genetically predisposed individuals, causing a malabsorptive syndrome. This review aims to conceptualize CD, describe its pathophysiology, highlight its epidemiology, detail associated clinical presentations, outline the diagnostic approach, and explain the treatment. To achieve this, works in English, Portuguese, and Spanish from the last five years were sought in the following databases: PubMed, LILACS, and SciELO, using the following keywords: “doença celíaca” and “celiac disease.” The search resulted in 175 articles, with 40 works chosen after screening and evaluation. The results revealed that CD affects approximately 1% of the global population, can be diagnosed at any age, but with more typical symptoms in children and atypical symptoms in adults. The pathogenesis involves the genetic factor HLA DQ2/DQ8, gluten ingestion, the formation of tissue transglutaminase antibodies, and an inflammatory reaction that damages the duodenum, impairing its absorptive function. Typical symptoms include chronic diarrhea, abdominal distension, weight loss, poor growth, and vitamin deficiencies. Atypical symptoms include dermatitis herpetiformis, refractory iron-deficiency anemia, delayed puberty, infertility, and neurological diseases. Diagnosis involves a suspected clinical picture, serologies for autoantibodies, genetic testing, and duodenal biopsy. Treatment is lifelong adherence to a gluten-free diet. Despite being well-characterized, CD remains underdiagnosed, and additional studies are needed.

Guidelines of the Italian societies of gastroenterology on the diagnosis and management of coeliac disease and dermatitis herpetiformis.

Coeliac disease and dermatitis herpetiformis are immune-mediated diseases triggered by the consumption of gluten in genetically predisposed individuals. These guidelines were developed to provide general practitioners, paediatricians, gastroenterologists, and other clinicians with an overview on the diagnosis, management and follow-up of coeliac patients and those with dermatitis herpetiformis. Guidelines were developed by the Italian Societies of Gastroenterology. Following a systematic literature review, the Grading of Recommendations Assessment, Development and Evaluation methodology was used to assess the certainty of the evidence. Statements and recommendations were developed by working groups consisting of gastroenterologists and a paediatrician with expertise in this field. These guidelines provide a practical guidance for the diagnosis, management and follow-up of coeliac patients and dermatitis herpetiformis in children and adults, both in primary care and in specialist settings. We developed four sections on diagnosis, gluten-free diet, follow-up and risk of complications in adults, one section focused on diagnosis and follow-up in children and one on the diagnosis and management of dermatitis herpetiformis. These guidelines may support clinicians to improve the diagnosis and management of patients with coeliac disease.

S1569 Gender-Based Association of Celiac Disease With Systemic Lupus Erythematosus and Other Rheumatologic Conditions: Analysis From National Inpatient Database

Introduction: Celiac disease is an immune mediated disorder of the digestive system that often presents with extraintestinal manifestations. The association of celiac disease with dermatitis herpetiformis, Type I Diabetes, IgA deficiency and chromosomal defects like Turner’s and Down’s syndrome is well known. However, the association of rheumatological diseases with Celiac disease remains controversial, especially based on gender. The aim of our study was to examine the association of rheumatological diseases with Celiac disease, stratified by gender. Methods: All adult hospitalized patients from January 2016 to December 2019 in nationwide inpatient sample (NIS) were captured. The study population included all patients with a diagnosis of Celiac disease using ICD-10 codes (International Classification of Diseases, tenth edition). Those without Celiac disease were included as the Control group. We identified patients with a diagnosis of SLE (Systemic Lupus Erythematosus), RA (Rheumatoid Arthritis), Fibromyalgia, Polymyalgia Rheumatica (PMR), Polymyositis/Dermatomyositis (PM/DM), Sjogren syndrome and Psoriatic Arthritis (PsA) using ICD-10 codes. We used linear regression for comparing continuous variables and Chi-square test for categorical variables. The association between Celiac disease and rheumatological conditions was analyzed using multivariate logistic regression model. Results: Our study included a sample size of 122,238,462 patients, of which 158,910 (0.13%) patients had Celiac disease and 122,079,552 (99.87%) patients did not. In the study group, highest risk was seen with Sjogren in both males (aOR=7.41) and females (aOR=3.93), with P< 0.001, followed by PM/DM (aOR=4.44 in males and aOR=2.11 in females, P-value < 0.001) compared to the control group. We also noted a higher risk of Celiac with SLE (aOR=1.72), RA (aOR=1.34), Fibromyalgia (aOR= 2.09), PMR (aOR=1.65) and PsA (aOR=2.71), all with P< 0.001 (Figure, Table). Similarly in males, greater risk was seen with SLE (aOR=2.51), RA (aOR=1.34), fibromyalgia (aOR=2.59), PMR (aOR=1.52), and PsA (aOR, 2.63). Conclusion: There is a significantly greater association of Rheumatological conditions with Celiac disease, more so in males than in females. Although, more studies are warranted to determine the causal relationship, knowledge about the association between them can help with early diagnosis and better management of these patients.Figure 1.: Graph 1 Table 1. - Association of Celiac disease with Rheumatologic diseases Rheumatologic diseases Females Males Unadjusted odds ratio Adjusted odds ratioa Unadjusted odds ratio Adjusted odds ratioa SLE* 2.49b 1.72b 2.89b 2.51b Rheumatoid Arthritis 1.83b 1.34b 1.55b 1.34b Fibromyalgia 3.06b 2.09b 3.44b 2.59b Polymyalgia Rheumatica 1.65b 1.35b 1.65b 1.52b Polymyositis/dermatomyositis 2.86b 2.11b 4.51b 4.44b Psoriatic arthritis 2.71b 1.57b 3.32b 2.63b Sjogren 6.68b 3.91b 10.25b 7.37b *SLE- Systemic Lupus Erythematosus.aAdjusted for age, race, Elixhauser score and other rheumatologic conditions.bsignificant and/or P<0.001.

S3373 Atypical GBS as the Presenting Symptom of Celiac Disease: Recognizing Extra-intestinal Presentations of Celiac Disease

Introduction: Celiac disease (CD) is an immune-mediated enteropathy in response to gluten that occurs in 1% of the population. Classic presenting symptoms include bloating, weight loss, and diarrhea. But, extraintestinal symptoms such as dermatitis herpetiformis, iron deficiency anemia, asymptomatic liver abnormalities, and more rarely, neurologic symptoms can be seen. We present a case of a patient who presented with worsening polyneuropathy and hepatitis leading to a final diagnosis of CD. Case Description/Methods: A 44-year-old male with history of recent left-chest shingles complicated by paralysis of right face and left leg weakness was initially admitted for suspected atypical Guillain Barre Syndrome (GBS) and treated with prednisone and a 5-day course of intravenous immunoglobulin (IVIG). The patient was readmitted a few weeks later with worsening neurologic status. A thorough infectious workup was unremarkable. The patient was also noted to have abnormal liver enzymes on prior admission that initially improved, but were noted to be worse on readmission up to ALP 1103, AST 634 and ALT 689. Given no prior history of liver disease, patient underwent a thorough workup including negative viral hepatitis, normal liver US, abdominopelvic CT showed scattered hypoattenuating lesions in the liver, one measuring up to 2.2 cm. He underwent a liver biopsy which demonstrated acute hepatitis with findings concerning for autoimmune versus drug-induced hepatitis. Autoimmune hepatitis panel was negative, however, he was found to have a tissue transglutaminase IgA >250 U/mL. Finally, he underwent upper endoscopy with biopsies that demonstrated findings consistent with celiac disease. Liver enzymes improved with gluten avoidance, but neurologic impairments persisted. Discussion: Atypical extra-intestinal symptoms at presentation of CD can result in diagnostic delay and permanent neurological disability if not treated. CD has been found in as many as 9% of patients with elevated liver enzymes. It is imperative not only to recognize neurologic presentations of CD but also the spectrum of possible liver impairments. Adherence to a strict gluten-free diet has been shown to improve symptoms of both neuropathy and liver enzyme abnormalities, but must be instituted quickly.

Leaky Gut and Dermatological Conditions

BACKGROUND Increased intestinal permeability, known as leaky gut syndrome, occurs when the intestinal lining of the gastrointestinal tract has been damaged. The influx of unwanted material in the bloodstream can induce an immune response releasing proinflammatory cytokines that have been thought to be correlated with dermatologic conditions. OBJECTIVE We aimed to evaluate the role of leaky gut in dermatological conditions. Methods PubMed and Embase were systematically searched through September 2020 for clinical studies that measured intestinal permeability in participants with a dermatological condition. RESULTS We identified 20 studies including participants with dermatitis herpetiformis, atopic dermatitis/eczema, systemic sclerosis, psoriasis, bullous pemphigoid, and pemphigus vulgaris/foliaceus. Atopic dermatitis was the most well studied. Studies utilized a range of measures of intestinal permeability, including sugar excretion tests and serum markers. For all disorders reviewed, except bullous pemphigoid and pemphigus vulgaris/foliaceus, there was evidence of increased intestinal permeability in the disease state compared with controls. LIMITATIONS The range of methodology across clinical studies makes it difficult to draw conclusions across results. In addition, studies tended to be small, and only one study included an intervention to target leaky gut syndrome. CONCLUSIONS We have found preliminary evidence of a relationship between leaky gut and certain dermatological conditions.

34524 The association between patient-reported outcomes skin flaring in autoimmune blistering diseases

Background: Autoimmune bullous diseases (ABDs) are chronic relapsing-remitting dermatologic diseases that exert significant impacts on quality of life (QOL). Using Skindex-16, we evaluated the impact of self-reported ABD flares on patients’ QOL. Methods: Skindex-16 is a dermatology-specific QOL measure that assesses skin disease impact on symptoms, emotions and functioning (range 0-100, higher scores = greater QOL impact). Demographic and clinical data for each visit were linked to PRO scores via manual chart review. Associations between PRO score and self-reported flare (“Are you flaring today? Yes/No”) were performed for each disease category (subepiderma, pemphigus, and dermatitis herpetiformis (DH)). Comparisons of Skindex-16 scores and individual item distributions by flaring status were compared using Somers’ D test. Odds of flare was calculated with univariable and multivariable logistic regression. Discrimination of indirect immunofluorescence lab values versus PRO scores was compared with c-index. Results: Between September 2016 and January 2020, we identified 193 patients with 317 visits. On average, patients took 2.44 minutes to complete the Skindex-16. Patients reported flaring at 29.0% of visits. Overall and individual item Skindex-16 were higher for all disease categories when flaring compared with non-flaring (P = .80 for all). Conclusions: ABDs have high morbidity. Flares are common and negatively impact QoL. These results highlight the clinical potential for serial Skindex-16 monitoring to signal clinical changes in patients with ABD.

31721 Dapsone for the treatment of neutrophilic dermatoses: A case series

Introduction: Systemic treatment for neutrophilic dermatoses (ND) is challenging due to limited options and available evidence. Dapsone is an oral sulfone drug with antimicrobial and anti-inflammatory effects. It inhibits the synthesis of dihydrofolic acid. Dapsone FDA-approved indications are leprosy and dermatitis herpetiformis. Nevertheless, dapsone has been used, off-label, in a wide variety of ND. We present a case series of 15 patients with several types of ND who received treatment with dapsone.

Bullous autoimmune dermatoses of the mucous membranes

Autoimmune bullous diseases (AIBD) comprise agroup of organ-specific autoimmune diseases which are characterised by the production of autoantibodies against adhesion molecules and structural proteins of skin and mucosae. Depending on the target protein, AIBD are classified into intraepidermal (pemphigus group) and subepidermal (pemphigoid group, epidermolysis bullosa acquisita, dermatitis herpetiformis) blistering disorders. Depending on the clinical entity, patients can develop blisters, pustules, erosions, and erythema on the skin and mucosae.

Bullous systemic lupus erythematosus in females.

Bullous systemic lupus erythematosus (BSLE) is a rare blistering presentation of systemic lupus erythematosus, typically affecting women with the highest incidence in those of African descent. The key pathogenic insult includes the formation of autoantibodies against type VII collagen, which weaken the basement membrane zone and lead to the formation of subepidermal blisters. The acute vesiculobullous eruptions in BSLE generally tend to affect photo-distributed areas, although they can arise unrelated to sun exposure (eg, mucous membranes, axillae). The bullae can arise from erythematous macules, inflammatory plaques, or previously normal skin. Their appearance can range from small, grouped vesicles reminiscent of lesions in dermatitis herpetiformis to large, tense blisters, similar to bullous pemphigoid. Internal organ involvement occurs in up to 90% of those affected. This mostly includes lupus nephritis (classes III–V, lifetime prevalence of up to 90%), arthralgias/arthritis, and cytopenias, while serositis and neuropsychiatric involvement are rare. First-line management with dapsone should be considered in mild disease with stable underlying systemic lupus erythematosus. As discussed in this review, the off-label use of rituximab (an anti-CD20 B-cell depleting agent) has been shown to be safe and effective in several refractory cases of BSLE unresponsive to dapsone, glucocorticoids, or steroid-sparing immunosuppressants.

164 Association between atopic dermatitis and celiac disease: A systematic review and meta-analysis

Atopic Dermatitis (AD) is one of the most prevalent complex chronic inflammatory skin diseases worldwide and has been associated with many different autoimmune disorders. Celiac disease (CD), an immune-mediated gluten-enteropathy linked with dermatitis herpetiformis (DH), has recently been among these disorders. To understand the relationship between these diseases, we performed the first meta-analysis looking at the association of CD in AD patients. A systematic review of PubMed, Cochrane Central, Embase, and Web of Science was performed on all studies prior to February 26, 2021 for keywords related to AD and CD.

The risk of renal comorbidities in celiac disease patients depends on the phenotype of celiac disease.

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. We investigated whether patients with different phenotypes of celiac disease are at higher risk of kidney diseases than age- and sex-matched references. 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. 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). 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.

Sex-differences in Gluten-free Dietary Adherence and Clinical Symptoms in Patients with Long-term Treated Dermatitis Herpetiformis.

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.

MO418: The Risk of Renal Co-Morbidities in Celiac Disease Patients Depends on the Phenotype of Celiac Disease

Abstract BACKGROUND AND AIMS Albeit the association between renal disorders and celiac disease is still somewhat contradictory, an elevated risk of kidney diseases in patients with celiac disease has been shown [1, 2, 3]. Only case reports have described the link between glomerulonephritis and dermatitis herpetiformis, a cutaneous manifestation of celiac disease [4]. This study aimed to evaluate whether patients with various phenotypes of celiac disease have an increased risk of renal co-morbidities compared with matched references. METHODS: The diagnoses of glomerulonephritis, diabetic nephropathy, interstitial nephritis and end-stage renal disease were collected from the Finnish National Hospital Discharge Register from 1970 to 2015 among 1440 celiac disease patients and 4296 age- and sex-matched references. Further, patients were divided into two cohorts, dermatitis herpetiformis and any other phenotype of celiac disease. We used Cox proportional-hazards model to compare the risk of renal co-morbidities between patients and reference individuals. RESULTS Celiac disease was positively associated with renal disorders even after adjusting for type 1 diabetes [hazard ratio (HR) 1.81, 95% confidence interval (CI) 1.18–2.76]. A 3-fold and 12-fold risk increase were seen for glomerulonephritis and IgA nephropathy, respectively. A similar risk increase for renal co-morbidities in dermatitis herpetiformis was not shown. CONCLUSIONS: Celiac disease was associated with an increased risk of renal co-morbidities, especially glomerulonephritis. The risk depends on the phenotype of celiac disease. The possible association between renal disorders and celiac disease should be investigated more in the future and kept in mind also in clinical practice.

Serological Biomarkers and Their Detection in Autoimmune Bullous Skin Diseases.

Autoimmune bullous diseases (AIBDs) are a group of skin-related disorders that involve damage to structures maintaining cell-cell adhesion, such as desmosomes and hemidesmosomes. Key AIBDs include pemphigus related diseases, pemphigoid related conditions, acquired epidermolysis bullosa (EBA), and dermatitis herpetiformis (DH). Each group of conditions exhibits characteristic clinical lesion patterns and is associated with specific autoantibodies targeting epidermal and dermal structures involved in cell-cell adhesion and skin integrity. Pemphigus diseases primarily target desmoglein (Dsg) 3 and Dsg1 proteins but several non-Dsg autoantibodies have also been linked to pemphigus.Pemphigoid diseases typically target bullous pemphigoid (BP)180 and BP230; EBA is associated with antibodies directed against anti-type VII collagen and DH by IgA autoantibodies against tissue transglutaminase and deaminated gliadin. Investigation into the serological biomarkers found in AIBDs have allowed the development of diagnostic assessments (i.e. tissue antibody detection and serological testing) based on the unique autoantibody profiles of a particular disease group. The methods for the detection and quantification of disease-associated autoantibodies continue to evolve and improve.

Oral immune-mediated disorders with malignant potential/association: An overview

A group of oral disorders or conditions, which may result from, or could be triggered by an abnormality in the normal immune response of an individual are known as oral immune-mediated disorders. Some of these disorders have malignant potential, while others are associated with malignancy. In this overview, we will discuss a few of the oral diseases (such as oral lichen planus, primary Sjogren's syndrome, systemic lupus erythematosus, dermatitis herpetiformis, and linear immunoglobulin A bullous dermatosis, to name a few), which are caused due to irregularity in the immune system and are either associated with malignancy or capable of undergoing malignant transforming, thereby increasing the morbidity and mortality rate.

Antibody Responses to Transglutaminase 3 in Dermatitis Herpetiformis: Lessons from Celiac Disease.

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.

Dermatitis herpetiformis Duhring as one of the forms of gluten-associated pathology: a review of the literature and a description of a clinical case

This review presents information on the prevalence, pathogenesis, clinical manifestations, diagnosis and treatment of Duhring’s dermatitis herpetiformis. Although the disease was first clinically described in 1884 by the American dermatologist L.A. Duhring, the study of its pathogenesis and the search for prognostic markers of its occurrence continue. Against the background of a significant expansion of ideas about gluten-dependent diseases and conditions, views on the mechanisms of autoimmune skin damage in dermatitis herpetiformis are detailed. A strong association with hereditary predisposition through the major human leukocyte histocompatibility complex (HLA) DQ2 and DQ8, and a role of epidermal transglutaminase as a major autoantigen in dermatitis herpetiformis are shown. The hypotheses explaining the decline in the incidence of dermatitis herpetiformis in recent decades against the background of increased and more effective serological screening and the resulting earlier diagnosis of celiac disease are commented on. A typical clinical picture of dermatitis herpetiformis, in which erythematous papules, plaques, vesicles are seen, usually clustered on the flexural surfaces of the extremities. Secondary elements are erosions, excoriations and crusts due to rupture of blisters and due to scratching caused by intense itching. A generally favourable prognosis for life and disease is shown with a gluten-free diet and the use of dapsone, glucocorticoids and, if these are ineffective, immunosuppressants. The authors describe a clinical case of the disease in an adolescent girl with a typical clinical history and characteristic rashes on the extensor surfaces of the limbs. The authors show that drug therapy without a gluten-free diet cannot be considered effective, and that the diet for dermatitis herpetiformis, like that for celiac disease, is lifelong. The growing understanding of gluten-associated pathology, which includes dermatitis herpetiformis, in recent decades has led to an intensive search for diagnostic and prognostic markers, as well as the development of ways to correct this group of diseases, including those not related to the lifelong elimination of cereal prolamines.