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Cureus Oct 2023Objectives The aim of this study was to describe the clinical, serological, and histopathological features of patients with dermatitis herpetiformis (DH) in Saudi...
Objectives The aim of this study was to describe the clinical, serological, and histopathological features of patients with dermatitis herpetiformis (DH) in Saudi Arabia. Methods We retrospectively reviewed the medical charts of all patients diagnosed with DH in the dermatology departments of National Guard Health Affairs (NGHA) hospitals in five different cities, from 2016 to 2022. We included patients who had been diagnosed by a dermatologist and had a combination of typical DH skin lesions, positive immunoglobulin A (IgA) on direct immunofluorescence (DIF), and/or positive tissue transglutaminase (tTG) IgA. Results A total of 11 patients were included. Their average age was 43.6 ± 12.5 years, and the ratio of females: males was 2.7: 1. Among the eight skin biopsies performed, IgA was detected on DIF in five patients. Seven out of nine patients (77.8%) had positive tTG IgA. Nine patients were managed with dapsone and a gluten-free diet (GFD); they had excellent responses within months. Conclusion The profiles of Saudi patients with DH were similar to those of Caucasian patients, but DH appears to be less common in Saudi Arabia. The high positive rates of tTG IgA make it an important tool for diagnosis in unclear cases. Dermatitis herpetiformis is likely associated with underlying gluten-sensitive enteropathy in Saudi patients.
PubMed: 38034235
DOI: 10.7759/cureus.48045 -
Nutrients Jan 2024Dermatitis herpetiformis is a cutaneous manifestation of celiac disease. Phenotyping of intraepithelial lymphocytes in the small bowel mucosa can strengthen the...
Dermatitis herpetiformis is a cutaneous manifestation of celiac disease. Phenotyping of intraepithelial lymphocytes in the small bowel mucosa can strengthen the diagnosis of celiac disease when it is not clear-cut. We aim to evaluate the usefulness of the intraepithelial lymphogram to confirm dermatitis herpetiformis in equivocal cases. We performed a retrospective multicenter study on patients diagnosed with dermatitis herpetiformis and collected data from the intraepithelial lymphogram assessed by flow cytometry. A total of 36 patients were analyzed in relation to the severity of intestinal damage (18 had non-atrophic mucosa) at baseline (N = 28) and/or after the adoption of a gluten-free diet (median follow-up of three years, N = 16). We observed that patients with atrophy more often had positive celiac serology ( = 0.019), celiac clinical symptoms ( = 0.018), and iron-deficiency anemia ( = 0.018), but the severity of skin damage was similar in both groups ( = 0.79). At baseline, increased TCRγδ cells were present in 94% of patients with atrophy and 67% with non-atrophic lesions ( = 0.13). After a gluten-free diet, increased TCRγδ cells persisted in 100% and 63% of cases, respectively ( = 0.21). We concluded that increased TCRγδ cells may be helpful in confirming the diagnosis of dermatitis herpetiformis in equivocal cases, even in patients who were started on a gluten-free diet.
Topics: Humans; Anemia, Iron-Deficiency; Atrophy; Celiac Disease; Data Collection; Dermatitis Herpetiformis; Retrospective Studies
PubMed: 38257124
DOI: 10.3390/nu16020232 -
Cureus Apr 2024Pemphigus herpetiformis (PH) is a rare autoimmune blistering disorder that typically presents in adults. However, its occurrence in paediatric patients, especially in...
Pemphigus herpetiformis (PH) is a rare autoimmune blistering disorder that typically presents in adults. However, its occurrence in paediatric patients, especially in very young children, is exceedingly rare. It presents with clinical features resembling dermatitis herpetiformis (DH) and immunologic characteristics similar to pemphigus, belonging to the group of intraepidermal autoimmune bullous diseases. We present the case of a three-year-old female with a history of annular and vesicular lesions on both forearms and legs. A skin biopsy revealed epidermal acanthosis, marked spongiosis, numerous intra-epidermal blisters, and exocytosis of eosinophils and neutrophils. A superficial perivascular lymphocytic infiltrate, accompanied by eosinophils and neutrophils, was also observed in the dermis. The diagnosis was also supported by direct and indirect immunofluorescence. The patient was treated with clobetasol ointment and dapsone, which showed significant improvement in the skin lesions. This case underscores the importance of considering PH in the differential diagnosis of vesicobullous diseases in children and the need for further research to elucidate its pathogenesis and optimal management.
PubMed: 38752034
DOI: 10.7759/cureus.58286 -
Frontiers in Medicine 2023Heat shock protein 90 alpha (Hsp90α) is one of the key intra- and extracellular chaperones responsible for the biological activity of various signaling molecules that...
Heat shock protein 90 alpha (Hsp90α) is one of the key intra- and extracellular chaperones responsible for the biological activity of various signaling molecules that are involved in (auto)immune-mediated inflammatory diseases. Recent epidemiologic data suggest that patients with atopic dermatitis (AD) are at risk for several autoimmune diseases, including dermatitis herpetiformis (DH), an extraintestinal manifestation of celiac disease (CD). In addition, pruritic diseases such as AD may be confused clinically with DH. In this study, we aimed to determine the role of circulating Hsp90α in patients with AD in relation to patients with DH, CD, and healthy controls. Using an enzyme-linked immunosorbent assay, levels of circulating Hsp90α were determined in serum samples derived from patients with AD ( = 31), DH ( = 26), CD ( = 15), and healthy controls ( = 55). Although serum concentrations of Hsp90α were similar between patients with DH, CD, and healthy controls, we found that serum levels of Hsp90α were significantly higher (mean value of 5.08-fold; < 0.0001) in patients with AD when compared to patients with DH. A cutoff value calculated as 2 × standard deviation above the mean concentration of Hsp90α in DH patients revealed that 83.9% (26/31) of AD patients were Hsp90α positive, whereas none of the DH patients (0/26) displayed such a positivity. This preliminary study suggests a distinct role for extracellular Hsp90α in the pathogenesis of AD compared to DH and its potential use in distinguishing AD from DH. Nevertheless, the potential role of the evaluation of extracellular Hsp90α for distinguishing between AD and DH is at present speculative and requires further and careful observations.
PubMed: 38249962
DOI: 10.3389/fmed.2023.1327144 -
Frontiers in Immunology 2023Autoimmune bullous diseases (AIBDs) are a group of rare cutaneous disorders affecting cornified skin and mucous membranes. They are characterized by tense or flaccid...
INTRODUCTION
Autoimmune bullous diseases (AIBDs) are a group of rare cutaneous disorders affecting cornified skin and mucous membranes. They are characterized by tense or flaccid blistering and erosions due to autoantibodies against desmosomal and hemidesmosomal structural proteins of the skin. This group of disorders can be divided into those of pemphigoid and those of pemphigus diseases. If left untreated, these autoimmune diseases can cause serious or even life-threatening complications such as loss of fluid, superinfections or impaired food intake. Due to modern standardized serological assays, the diagnosis of AIBDs can usually be confirmed in combination with their clinical appearance. Whereas for a long time corticosteroids were the major players in the treatment of these diseases, with the approval of rituximab and other immunosuppressive agents, the therapy has increasingly improved.
METHODS
In this study, we aimed to investigate epidemiologic and clinical features as well as diagnostics and therapy of bullous autoimmune diseases in Middle Franconia, a governorate within the German federal state of Bavaria. Patients diagnosed or treated because of a AIBDs between 01.04.2013 and 31.03.2019 at the dermatological department of the university hospital Erlangen were included in this retrospective study (n = 242). Patients were either diagnosed for the first time (n=176) or the diagnosis has been confirmed (n=66) at the department. The respective incidence was calculated among the 176 subjects who had been diagnosed at the center in this period. Data was taken from patient records and analyzed with Microsoft® Excel. The evaluation included the diagnoses of pemphigus vulgaris (PV), pemphigus foliaceus (PF), bullous pemphigoid (BP), mucous membrane pemphigoid (MMP), linear IgA dermatosis (LAD), epidermolysis bullosa acquisita (EBA), and dermatitis herpetiformis (DH).
RESULTS
This study shows that the incidence of each AIBDs in Middle Franconia is low and comparable (PV, PF, LAD, EBA) or lower (BP, MMP, DH) than in other studies and regions. BP is the most common newly diagnosed AIBD in Middle Franconia.
DISCUSSION
Due to the chronic and sometimes severe course of AIBDs, repeated in-house treatments are often necessary. To date, mainly topically and systemically applied corticosteroids in combination with immunomodulators are used as first-line therapy.
Topics: Humans; Retrospective Studies; Pemphigoid, Bullous; Autoimmune Diseases; Skin Diseases, Vesiculobullous; Pemphigus; Linear IgA Bullous Dermatosis; Epidermolysis Bullosa Acquisita; Adrenal Cortex Hormones
PubMed: 37881435
DOI: 10.3389/fimmu.2023.1256617 -
Nutrients Dec 2023Celiac disease (CD) is an immune-mediated systemic gluten-related disorder characterized by a wide spectrum of intestinal and extra-intestinal manifestations, including... (Review)
Review
Celiac disease (CD) is an immune-mediated systemic gluten-related disorder characterized by a wide spectrum of intestinal and extra-intestinal manifestations, including damage to cutaneous and connective tissue. We report a rare case of chronic severe dermatitis involving connective tissue and cutaneous vascular vessels as the main clinical presentation of undiagnosed seronegative gluten disorder. A gluten-free diet dramatically improved the intestinal and cutaneous clinical damage in the patient. Pitfalls and the steps of differential diagnosis are described. We also review the literature regarding studies of CD and connective tissue diseases to extend the knowledge of these rare associations. We propose a practical diagnostic approach in suspected CD in autoimmune cutaneous disorders.
Topics: Humans; Celiac Disease; Skin Diseases; Dermatitis; Glutens; Autoimmune Diseases
PubMed: 38201912
DOI: 10.3390/nu16010083 -
Indian Journal of Dermatology 2024Gluten, a polypeptide hapten, found in many cereals such as barley, wheat, rye, oats, and others, has been recently implicated in a range of cutaneous disorders ranging... (Review)
Review
Gluten, a polypeptide hapten, found in many cereals such as barley, wheat, rye, oats, and others, has been recently implicated in a range of cutaneous disorders ranging from chronic plaque psoriasis through psoriatic arthritis, urticaria (chronic as well as paediatric onset), and angioedema to lichen planus, vitiligo, and rosacea. The evidence for them is still not well reviewed. To generate evidence for the causal role of gluten in various dermatological disorders. The Pubmed, MedLine, and EMBASE databases were searched using the keywords "Gluten" and one of the dermatoses, namely, "Atopic Dermatitis", "Vasculitis", "Psoriasis", "Psoriatic Arthritis", "Acne", "Alopecia Areata", and "Immunobullous disorders". All articles published in English for which free full text was available were taken into consideration. The search strategy returned in a total of 1487 articles which were screened for relevance and elimination of duplicates. Ultimately, around 114 articles were deemed suitable. The data were extracted and presented in the narrative review format. A simple and cost-effective solution to many of these chronic and lifelong conditions is to restrict gluten in the diet. However, the dermatologist would do well to remember that in the vast majority of dermatological disorders including the ones listed here, gluten restriction is not warranted and can even lead to nutritional deficiencies. The evidence varied from Grade I for some disorders like psoriatic arthritis to Grade IV to most disorders like acne, vitiligo, vasculitis, and atopic dermatitis. Herein, we review the evidence for each of these conditions and make practical recommendations for gluten restriction in them.
PubMed: 38841247
DOI: 10.4103/ijd.ijd_815_22 -
Revista Alergia Mexico (Tecamachalco,... Feb 2024Brief erythematous-papular skin rashes suggest the diagnosis of urticaria; However, it may be another type of dermatitis, and complementary examinations must be carried...
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.
Topics: Humans; Middle Aged; Female; Chronic Urticaria; Dermatitis Herpetiformis; Pruritus; Diagnosis, Differential; Dapsone
PubMed: 38683068
DOI: 10.29262/ram.v71i1.1245 -
Italian Journal of Dermatology and... Feb 2024The oral mucosa can be involved in a wide variety of mucocutaneous conditions that may present primarily in the mouth or affect other cutaneous or mucosal sites. Many of... (Review)
Review
The oral mucosa can be involved in a wide variety of mucocutaneous conditions that may present primarily in the mouth or affect other cutaneous or mucosal sites. Many of these conditions are immune mediated and typically present as inflammatory mucosal pathology. Patients experiencing such conditions usually seek medical evaluation and treatment due to the associated pain and discomfort, and occasionally taste disturbance or dysphagia and the overall deterioration in the oral health-related quality of life. These conditions share some common features and there could be some overlap in their clinical presentation, which can lead to delays in diagnosis and proper management of patients. Clinicians dealing with such disorders, including dermatologists, need to be aware of the oral manifestations of mucocutaneous conditions, their clinical features, underlying mechanisms, diagnostic approaches, and treatment options, as well as the recent advances in the research on these conditions. This review provides a comprehensive, evidence-based reference for clinicians, with updated insights into a group of immune mediated conditions known to cause oral mucosal pathology. Part one will cover oral lichen planus, erythema multiforme and systemic lupus erythematosus, while part two will cover recurrent aphthous stomatitis, pemphigus vulgaris and mucous membrane pemphigoid, in addition to the less common disorders linear IgA disease, dermatitis herpetiformis and epidermolysis bullosa.
Topics: Humans; Mouth Mucosa; Mouth Diseases; Quality of Life; Pemphigus; Stomatitis, Aphthous
PubMed: 38345290
DOI: 10.23736/S2784-8671.23.07690-9 -
Alimentary Pharmacology & Therapeutics Mar 2024Data on mortality in coeliac disease are contrasting. (Review)
Review
BACKGROUND
Data on mortality in coeliac disease are contrasting.
AIMS
To systematically review the literature on all-cause and cause-specific mortality in coeliac disease compared to the general population, and evaluate differences across clinical phenotypes, geographical regions, and over time.
METHODS
We searched PubMed and Embase from 1 January 1970 to 31 December 2022 for eligible studies reporting on all-cause and cause-specific mortality in coeliac disease compared to the general population or controls. The protocol was registered on Open Science Framework (https://doi.org/10.17605/OSF.IO/852DN).
RESULTS
We included 25 studies. All-cause mortality (HR 1.16, 95% CI 1.05-1.27, I = 89%), mortality due to malignancies (HR 1.21, 95% CI 1.08-1.36, I = 65%) and respiratory disease (HR 1.39, 95% CI 1.04-1.86, I = 76%) were increased. Mortality due to non-Hodgkin lymphoma (HR 10.14, 95% CI 2.19-46.88, I = 96%) was markedly increased. Mortality significantly decreased in recent decades: 1989-2004 (HR 1.61, 95% CI 1.27-2.03, I = 91%), 2005-2014 (HR 1.16, 95% CI 0.99-1.36, I = 89%), 2015-2022 (HR 1.19, 95% CI 1.05-1.35, I = 93%). All-cause mortality was not increased in dermatitis herpetiformis (HR 0.85, 95% CI 0.73-0.99, I = 40%) and undiagnosed coeliac disease (HR 1.09, 95% CI 0.95-1.25, I = 0%). Mortality was increased in the UK (HR 1.23, 95% CI 1.03-1.47, I = 91%) but not Scandinavia (HR 1.01, 95% CI 0.91-1.13, I = 81%). Limitations include high heterogeneity and lack of data for many countries.
CONCLUSION
Mortality in coeliac disease is increased, predominantly due to malignancies-particularly non-Hodgkin lymphoma-although differing significantly across disease phenotypes. Mortality of patients with coeliac disease has significantly decreased in recent decades. These results may influence diagnosis and management.
Topics: Humans; Celiac Disease; Lymphoma, Non-Hodgkin; Neoplasms
PubMed: 38204404
DOI: 10.1111/apt.17867