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Dermatitis Herpetiformis: From the Genetics to the Development of Skin Lesions
Diletta Bonciani,Alice Verdelli,Veronica Bonciolini,Antonietta D'Errico,Emiliano Antiga,Paolo Fabbri,Marzia Caproni
Clinical and Developmental Immunology , 2012, DOI: 10.1155/2012/239691
Abstract: Dermatitis herpetiformis (DH) is a rare autoimmune disease linked to gluten sensitivity with a chronic-relapsing course. It is currently considered to be the specific cutaneous manifestation of celiac disease (CD). Both conditions are mediated by the IgA class of autoantibodies, and the diagnosis of DH is dependent on the 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. This paper describes these different factors and discusses the known mechanism that lead to the development of skin lesions.
Gluteal skin is not an optimal biopsy site for direct immunof luorescence in diagnostics of dermatitis herpetiformis. A report of a case of dermatitis herpetiformis Cottini Skóra okolicy po ladkowej nie jest optymalnym miejscem biopsji do bezpo redniego badania immunofluorescencyjnego w diagnostyce opryszczkowatego zapalenia skóry. Opis przypadku opryszczkowatego zapalenia skóry w odmianie Cottiniego
Anna Lutkowska,Pawe? Pietkiewicz,Joanna Sulczyńska-Gabor,Justyna Gornowicz
Clinical Dermatology , 2009,
Abstract: Dermatitis herpetiformis (DH) isan autoimmunebullous dermatosisconnected with gluten-sensitiveenteropathy. Direct immunofluorescence test (DIF) of nonlesional skin remainsdefinitivelaboratory test fordiagnosingthisdisease. We present here a case of DH Cottini, inwhom DIF of gluteal skin was negative, but DIFof perilesional knee skin revealed IgA deposits characteristic for DH. On the ground of literature data and our own experience we think that in patients suspected of having DH, in whom there were and there are no lesions on buttocks (such as in cases of DH Cottini) DIF should not be performed on gluteal skin. Still, in DH cases in whom lesions are on buttocks, gluteal skin should still be regarded as an appropriate biopsy site for performing DIF.
The Expression of Selected Proapoptotic Molecules in Dermatitis Herpetiformis
Zebrowska Agnieszka,Erkiert-Polguj Anna,Wagrowska-Danilewicz Malgorzata,Danilewicz Marian,Sysa-Jedrzejowska Anna,Anna Cynkier,Waszczykowska Elzbieta
Clinical and Developmental Immunology , 2012, DOI: 10.1155/2012/178340
Abstract: The role of the process of apoptosis is investigated in the pathogenesis of many autoimmune diseases; however at present, there is not much information about its role in dermatitis herpetiformis. Skin biopsies were taken from 18 DH patients and from 10 healthy subjects. The localization and expression of Bax, Fas, FasL, TRAIL, TRAIL-R in skin lesions, and perilesional skin were studied by immunohistochemistry. Expression of Bax, Fas, and Fas ligand was detected in the keratinocytes in skin biopsies from DH patients. Expression of TRAIL and TRAIL receptor was confirmed in epidermis, infiltration cells, and some fibroblasts. The expression of examined molecules in biopsies from healthy people was observed only in single cells. There were statistically significant differences between lesional, perilesional, and healthy skin of control group in Bax expression analysis and between lesional skin and control group in Fas, FasL, and TRAIL expression. There were statistically significant differences between control group and perilesional skin in Bax and FasL expression. Our results show that selected proapoptotic molecules may take part in pathogenesis of dermatitis herpetiformis, but the role of apoptosis in this process is not clear.
Recent Advances in Dermatitis Herpetiformis
Kimiko Nakajima
Clinical and Developmental Immunology , 2012, DOI: 10.1155/2012/914162
Abstract: Dermatitis herpetiformis is an autoimmune bullous disease that is associated with gluten sensitivity which typically presents as celiac disease. As both conditions are multifactorial disorders, it is not clear how specific pathogenetic mechanisms may lead to the dysregulation of immune responses in the skin and small bowel, respectively. Recent studies have demonstrated that IgA and antibodies against epidermal transglutaminase 3 play an important role in the pathogenesis of dermatitis herpetiformis. Here, we review recent immunopathological progress in understanding the pathogenesis of dermatitis herpetiformis.
Dermatitis herpetiformis bodies and autoantibodies to noncutaneous organs and mitochondria in dermatitis herpetiformis  [cached]
Ana Maria Abreu Velez,Hong Yi,Julia Griffin Girard,Zhe Jiao
Our Dermatology Online , 2012,
Abstract: Introduction: The precise nature of the previously described dermatitis herpetiformis bodies remains unknown.Aims: Our study was conducted to investigate the nature of dermatitis herpetiformis bodies in the skin in 7 cases of dermatitis herpetiformis, and to search for the presence of autoantibodies in other organsMethods: We utilized clinical, histopathologic, and immunologic methods to evaluate these patients.Results: Dermatitis herpetiformis bodies were found to be comprised of an amalgamation of immunoglobulins A and M, as well as molecules reactive with antibodies to armadillo repeat gene deleted in velo-cardio-facial syndrome, desmoplakins 1 and 2, and plakophilin 4. In addition, we found immunologic colocalization with selected autoantibodies associated with mitochondria in the skin, heart, kidney, and peripheral nerves. The dermatitis herpetiformis bodies did not demonstrate immunologic colocalization with tissue/epidermal transglutaminase.Conclusion: The complete biochemical nature of dermatitis herpetiformis bodies requires further characterization. Dermatitis herpetiformis bodies in these patients appear to be distinctly different than cytoid bodies. Further studies are required to determine if the antibodies to noncutaneous organs are pathogenic, and/or contribute to systemic morbility in dermatitis herpetiformis patients.
The Imbalance Between Metalloproteinases and Their Tissue Inhibitors Is Involved in the Pathogenesis of Dermatitis Herpetiformis  [PDF]
Agnieszka Zebrowska,Joanna Narbutt,Anna Sysa-Jedrzejowska,Jozef Kobos,Elzbieta Waszczykowska
Mediators of Inflammation , 2005, DOI: 10.1155/mi.2005.373
Abstract: Dermatitis herpetiformis (DH) is a subepidermal autoimmune disease characterized by skin and intestinal lesions consistent with coeliac disease. There are also some data that metalloproteinases (MMPs) are involved in the development of skin lesions in DH, however their exact role in this process is not fully understood. The aim of the study was to investigate whether MMPs and their inhibitors are involved in pathogenesis of DH. Skin biopsies were taken from 13 patients with active DH and from 10 healthy subjects. The localization and expression of MMPs and TIMPs were examined by immunohistochemistry. MMPs expression was detected in basal keratinocytes and in the whole epidermis in all of the DH subjects. Neutrophils in microabscesses and in blister fluid were also positive for MMPs. Expression of TIMPs was moderate or weak in all examined biopsies. Our results allow us to conclude that imbalance between these enzymes takes an important role in the pathogenesis of DH.
Dermatitis herpetiformis intolerant to dapsone in Aids  [cached]
Krishna K,Kavitha K
Indian Journal of Dermatology, Venereology and Leprology , 1999,
Abstract: A 35-year-old man with AIDS and pulmonary tuberculosis presented with lesions suggestive of dermatitis herpetiformis and intolerance to dapsone. He was managed successfully with a combination of nicotinamide 200 mg/day and indomethacin 75 mg/day, topical steroids and gluten free diet.
Low Strength of Correlation between the Intensity of Neutrophil Elastase Expression in Lesional Skin and the Level of Serum IgA Antibodies to Epidermal Transglutaminase in Dermatitis Herpetiformis
M. Bowszyc-Dmochowska, A. Seraszek, E. Kaczmarek, J. GornowiczM. Dmochowskilamina lucida
The Open Autoimmunity Journal , 2009, DOI: 10.2174/1876894600901010001]
Abstract: Whereas it has been shown that neutrophil elastase (NE) is a crucial enzyme degrading the dermal-epidermal junction (DEJ) in bullous pemphigoid (BP), experimental studies on the role of NE in dermatitis herpetiformis (DH), a disease in which, as in BP, an intra-lamina lucida blister is formed, are scanty. The aim of this study was to analyse whether there is a correlation between levels of serum IgA antibodies to the epidermal transglutaminase (TG3), an enzyme believed to be the autoantigen of DH, and expression of NE in lesional skin in DH. A series of 21 consecutive patients with DH was studied. The levels of IgA antibodies to TG3 in sera were calculated with ELISA. The expressions of NE were examined with immunohistochemical technique in sections of lesional skin using a mouse monoclonal antibody to human NE. The digital microscopic image analysis with the appropriate software was then used to measure intensities of NE expression. The correlation between the intensity of NE expression in lesional skin and the level of serum IgA antibodies to TG3 in DH was of low strength. Thus, it is speculated that in DH the engagement of IgA autoantibodies to the enzyme, TG3, on cutaneous neutrophils might not be a principal stimulus to releasing NE, the enzyme known to degrade DEJ in subepidermal blistering diseases with autoimmunity to DEJ structural proteins.
Newly Described Clinical and Immunopathological Feature of Dermatitis Herpetiformis
Veronica Bonciolini,Diletta Bonciani,Alice Verdelli,Antonietta D'Errico,Emiliano Antiga,Paolo Fabbri,Marzia Caproni
Clinical and Developmental Immunology , 2012, DOI: 10.1155/2012/967974
Abstract: Dermatitis herpetiformis (DH) is an inflammatory cutaneous disease with typical histopathological and immunopathological findings clinically characterized by intensely pruritic polymorphic lesions with a chronic-relapsing course. In addition to classic clinical manifestations of DH, atypical variants are more and more frequently reported and histological and immunological are added to them, whereas the impact on quality of life of patients with DH is increasingly important to a certain diagnosis. The aim of this paper is to describe all the possible clinical, histological, and immunological variants of DH in order to facilitate the diagnosis of a rare disease and, therefore, little known.
Application of concentrated deep sea water inhibits the development of atopic dermatitis-like skin lesions in NC/Nga mice  [cached]
Bak Jong-Phil,Kim Yong-Min,Son Jeonghyun,Kim Chang-Ju
BMC Complementary and Alternative Medicine , 2012, DOI: 10.1186/1472-6882-12-108
Abstract: Background Mineral water from deep-sea bedrock, formed over thousands of years, is rich in minerals such as Ca, Mg, Na, K, Fe and others. Our present study was to investigate the preventive effects of natural deep-sea water on developing atopic dermatitis (AD). Methods We elicited AD by application of DNCB (2,4-dinitro-chlorobezene) in Nc/Nga mouse dorsal skin. Deep Sea water (DSW) was filtered and concentrated by a nanofiltration process and reverse osmosis. We applied concentrated DSW (CDSW) to lesions five times per week for six weeks, followed by evaluation. 1% pimecrolimus ointment was used as positive control. The severity of skin lesions was assessed macroscopically and histologically. Levels of inflammatory mediators and cytokines in the serum were detected by Enzyme-linked immunosorbent assay (ELISA) and the levels of CD4+ and CD8+ spleen lymphocytes were determined by flow cytometry analysis. Results DNCB-treated mice showed atopic dermatitis-like skin lesions. Treatment of mice with CDSW reduced the severity of symptoms in the skin lesions, including edema, erythema, dryness, itching, and transepidermal water loss (TEWL). Histological analyses demonstrated that epidermal thickness and infiltration of inflammatory cells were decreased after CDSW treatment. Given these interesting observations, we further evaluated the effect of CDSW on immune responses in this AD model. Treatment AD mice with CDSW inhibited up-regulation of IgE, histamine, and pro-inflammatory cytokines in the serum. Also, the CD4+/CD8+ ratio in spleen lymphocyte was down-regulated after treatment with CDSW. Finally, cytokines, especially IL-4 and IL-10 which are important for Th2 cell development, were reduced. Conclusions Our data suggests that topical application of CDSW could be useful in preventing the development of atopic dermatitis.
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