Dermatitis herpetiformis in an African woman.

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.

The Role of Intereukin-31 in Pathogenesis of Itch and Its Intensity in a Course of Bullous Pemphigoid and Dermatitis Herpetiformis.

Itch which is one of the major, subjective symptoms in a course of bullous pemphigoid and dermatitis herpetiformis makes those two diseases totally different than other autoimmune blistering diseases. Its pathogenesis is still not fully known. The aim of this research was to assess the role of IL-31 in development of itch as well as to measure its intensity. Obtained results, as well as literature data, show that lower concentration of IL-31 in patients' serum may be correlated with its role in JAK/STAT signaling pathway which is involved in development of autoimmune blistering disease. Intensity of itch is surprisingly huge problem for the patients and the obtained results are comparable with results presented by atopic patients.

Dermatitis herpetiformis and bone mineral density: analysis of a French cohort of 53 patients.

The characteristics of patients with dermatitis herpetiformis (DH) in France is poorly documented. Furthermore, the risk of fractures and bone mineral density (BMD) in DH remain under-described, and recommendations for systematic screening for osteoporosis in DH are lacking. To describe the characteristics of DH in a large French cohort and evaluate the association between BMD and features of osteoporosis. Patients were recruited from the French Association of Gluten Intolerants (AFDIAG) and a single university dermatology department. A telephone questionnaire was used to record features of DH, history of fractures, calcium intake, treatment, and the gluten-free diet (GFD). Serum calcium and 25(OH) vitamin D3+D2 levels, as well as BMD, were measured. We included 53 patients (27 men) with a median age of 49 years (range: 23-86). Median disease duration before inclusion was 14 years (range: 2-55); 51 patients (96%) were adherent to a GFD and had no digestive symptoms. Overall, 18 (34%) had a history of fractures; 16 high-velocity (traumatic) and two low-velocity (non-traumatic). Mean BMD, measured in 48 patients, was normal (femoral neck: 0.956 ± 0.210 g/cm2; lumbar spine: 1.091 ± 1.199 g/cm2). In all, 18 patients (38%) had osteopenia and one (2%) osteoporosis. T-score for bone density did not differ with and without fractures. Calcium intake and serum calcium level were normal in all patients. Screening for osteoporosis does not appear to be mandatory for DH patients with good adherence to a GFD and without digestive symptoms or additional risk factors of osteoporosis.

Immunoexpression of IgA receptors (CD89, CD71) in dermatitis herpetiformis.

INTRODUCTION: The role of IgA receptors in dermatitis herpetiformis (DH) pathogenesis is still unknown. CD89 and CD71 may be associated with immune response during DH development. The purpose of this study was to perform semiquantitative analysis of simultaneous immunoexpression of CD89 and CD71 in DH and IgA/neutrophil-mediated non-DH dermatoses (IgAN) in relation to specific IgA autoantibodies/antibodies (tissue and epidermal transglutaminases, nonapeptides of gliadin - eTG/tTG/npG) as well neutrophil activation via the release of neutrophil elastase (NE). MATERIAL AND METHODS: In total, 48 patients were studied. The study was conducted on skin lesions and sera obtained from DH and IgAN patients. DH and IgAN served as mutually positive control groups. We used immunohistochemical technique with semiquantitative digital morphometry and ELISA to measure serum levels of anti-eTG/tTG/npG IgA. RESULTS: CD89 showed a significantly higher expression in DH than in IgAN. CD71 was overexpressed in DH and IgAN. CD89 immunoexpression correlated negatively with CD71 in IgAN. A positive correlation was revealed between CD89 immunoexpression and anti-npG IgA in DH. No statistically significant correlations were found in DH between the CD89/CD71 and NE immunoexpression, between CD71 immunoexpression and anti-tTG/eTG/npG IgA, or between CD89 immunoexpression and anti-eTG/tTG IgA serum levels. CONCLUSIONS: CD89 is probably a key IgA Fc receptor in DH development, where it is associated with immune response to gluten. CD71 may be linked with inflammation in DH and IgAN. We suggest that interaction between CD89 and CD71 can modulate the inflammation in IgAN.

Gastrointestinal Symptoms Increase the Burden of Illness in Dermatitis Herpetiformis: A Prospective Study.

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

Associations of coeliac disease with coronary heart disease and cerebrovascular disease: A systematic review and meta-analysis.

AIMS: Clinical experience suggests that atherosclerotic disease is common in individuals with coeliac disease, but epidemiological studies have had contradicting findings. To summarise the currently available evidence, we systematically reviewed and analysed observational studies of the association of coeliac disease or dermatitis herpetiformis with coronary heart disease (CHD) or stroke. DATA SYNTHESIS: We searched for studies comparing CHD or stroke outcomes with individuals with and without coeliac disease or dermatitis herpetiformis. Three investigators independently searched electronic databases, identified relevant studies and extracted data. Study-specific results were combined in random-effects meta-analyses, and heterogeneity was quantified using the I(2) statistic and meta-regression. Twenty-one studies were included in our systematic review and 18 in the meta-analyses. For CHD, the pooled hazard ratio for incident disease was 1.05 (95% confidence interval (CI): 0.93, 1.19) and the overall standardised mortality ratio was 1.21 (0.99, 1.49). For stroke and brain haemorrhage, the corresponding estimates were 1.10 (95% CI: 1.00, 1.21) and 1.43 (0.97, 2.10), respectively. There was moderate to considerable heterogeneity among the study-specific estimates. In addition, many estimates were based on small numbers of outcomes and they had limitations in terms of adjustment for potential confounders. CONCLUSION: Our meta-analyses lend some support to an association between coeliac disease and CHD or cerebrovascular disease, but the evidence base was heterogeneous and had limitations. Our systematic review highlighted a need in this area for adequately powered prospective studies with appropriate adjustment for potentially confounding factors.

Pemphigus herpetiformis: from first description until now.

Pemphigus herpetiformis is one of the less common forms of pemphigus, first introduced by Jablonska and colleagues in 1975. This autoimmune bullous disease combines the clinical features of dermatitis herpetiformis and the immunologic characteristics of pemphigus. The target autoantigen is usually desmoglein 1 (or less frequently desmoglein 3), although recently it has become increasingly obvious that patients with pemphigus herpetiformis also demonstrate autoantibodies against desmocollin. Here, we summarize reported cases of pemphigus herpetiformis and describe current knowledge considering epidemiology, clinical manifestations, histologic findings, immunopathology, pathophysiologic concepts, associated diseases, and treatment of this rare disorder.

Pathophysiology of dermatitis herpetiformis: a model for cutaneous manifestations of gastrointestinal inflammation.

Dermatitis herpetiformis (DH) is an autoimmune blistering skin disease in which antigen presentation in the gastrointestinal mucosa results in cutaneous IgA deposition and distinct, neutrophil-driven cutaneous lesions. Our findings suggest that the qualitatively different immune response to gluten in the intestinal mucosa of patients with DH results in minimal clinical symptoms, allowing the continued ingestion of gluten and the eventual development of DH. Our model may provide a new way to understand the pathogenesis of other skin diseases associated with gastrointestinal inflammation such as pyoderma gangrenosum or erythema nodosum, or explain association of seronegative inflammatory arthritis with inflammatory bowel disease.

Dermatitis herpetiformis. Part I. Epidemiology, pathogenesis, and clinical presentation.

Dermatitis herpetiformis (DH) is an autoimmune disease that is linked to gluten sensitivity and has a clear relationship to celiac disease. Both conditions are mediated by the IgA class of autoantibodies and the diagnosis of DH is dependent on detection of granular deposits of IgA in the skin. There is an underlying genetic predisposition to the development of DH but environmental factors are also important. Typically, young adults present with excoriations only, as the severe pruritus effectively destroys any primary lesions. Based upon our experience with DH and a comprehensive literature review, we provide an update of DH epidemiology, pathophysiology, and clinical presentation.

[Dermatitis herpetiformis: a review]. / Dermatite herpétiforme: revue de la littérature.

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

Remission in dermatitis herpetiformis: a cohort study.

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

Disnea y cianosis acra en paciente con dermatitis herpetiforme / No disponible

No disponible

A case of stiff-person syndrome, type 1 diabetes, celiac disease and dermatitis herpetiformis.

Antibodies against glutamic acid decarboxylase (GAD) are involved in the pathophysiology of stiff-person syndrome (SPS) and type 1 diabetes. GAD catalyses the conversion of glutamate to gamma-aminobutyric acid (GABA). GABA acts as a neurotransmitter between neurones, while in pancreatic beta cells it plays an integral role in normal insulin secretion, hence the clinical presentation of muscular spasms in SPS and insulin deficiency in diabetes. Despite this apparent major overlap in pathophysiology, SPS only rarely occurs in individuals with type 1 diabetes. We report the case of a 41-year-old man presenting with a simultaneous diagnosis of both these conditions. His case is unusual in that it is the first reported case in the literature of these conditions occurring in someone with celiac disease (CD) and dermatitis herpetiformis. We discuss why SPS and type 1 diabetes co-exist in only a minority of cases and speculate on the underlying mechanism of the association with CD and dermatitis herpetiformis in our patient.