The pathogenesis of dermatitis herpetiformis sciencedirect. Hla, play an important role in the pathogenesis of dh 23. Dermatitis herpetiformis dh is a subepidermal bullous disease characterized by chronic recurrence of itchy, erythematous papules, urticarial wheals and grouped vesicles that appear symmetrically on the extensor surfaces, buttocks and back. An evaluation of the diagnostic criteria br j dermatol 90. There was no history of photosensitivity or any other aggravating factors. The pathogenesis of dermatitis herpetiformis in the skin is related to the pathogenesis of celiac disease in the gut. Keywords autoimmune bullous diseases, celiac disease, tissue. She was seen in shengzhan by traditional chinese and western doctors.
Marcos vinicius clarindo, adriana tomazzoni possebon. What is the pathophysiology of dermatitis herpetiformis. A third major factor that appears to be relevant in the pathogenesis of dermatitis herpetiformis is the strong as sociation of the disease with specific hla antigens. Hormonal factors may also play a role in the pathogenesis of dermatitis herpetiformis, and reports describe dermatitis herpetiformis induced by treatment with leuprolide acetate, a gonadotropinreleasing hormone analog 26. Both are mediated by iga autoantibodies, triggered by the ingestion of gluten. Affected patients typically develop intensely pruritic inflammatory papules and vesicles on the forearms, knees, scalp, or buttocks picture 1ag. Pastore et al pathogenesis of atopic dermatitis saveria pastore1, francesca mascia1, maria laura giustizieri1, alberto giannetti2 and giampiero girolomoni1 1 laboratory of immunology, istituto dermopatico dellimmacolata, irccs, rome, italy, 2department of dermatology, university of. Like celiac disease, dermatitis herpetiformis is more common in genetically predisposed individuals carrying either the. Attempts to address the pathophysiology of dermatitis herpetiformis must take the following characteristics into account.
The prevalence of hladq2 and dq8 is the same as in celiac disease, supporting the concept that dermatitis herpetiformis is a. Dermatitis herpetiformis dh is a subepidermal bullous disease characterized by chronic. Dermatitis herpetiformis prevalence varies across countries. This study aimed to investigate the safety and longterm quality of life and health effects of oat consumption in 312 longterm treated dh patients. Systemic lupus erythematosus and dermatitis herpetiformis. Dermatitis is a general term for skin inflammation.
The diagnosis and treatment of dermatitis herpetiformis ccid. In a gfd, wheat, rye and barley should be strictly avoided, but the role of oats is more controversial. The pathophysiology of dh likely involves a complex interplay between autoimmune factors, such as human leukocyte antigen hla predisposition, genetics, and environment. Dermatitis, also known as eczema, is a group of diseases that result in inflammation of the skin. 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. What is the role of food allergies in the pathogenesis of. Dermatitis herpetiformis dh is a blistering skin disease in which the patients have mostly subclinical gluten sensitive enteropathy, i. Dermatitis herpetiformis dh is a chronic autoimmune blistering skin condition, characterised by blisters filled with a watery fluid that is intensely itchy. Dermatitis herpetiformis is characterized by an itchy blistering rash. This is a form of nonige cellmediated hypersensitivity related to celiac disease. Ho case summary history a 23year old chinese woman presented with a three year history of recurrent blistering eruption that is associated with mild itching. There is increasing evidence that, as in systemic lupus erythematosus sle, deposition of immune complexes plays a role in the pathogenesis of dermatitis herpetiformis dh.
The imbalance between metalloproteinases and their tissue. Strober, dermatology and metabolism branches, national cancer institute, national institutes of health, bethesda, maryland, u. Onset of dermatitis herpetiformis is usually early to middle adult life but can occur in children and later in life. Introduction pruritus is one of the main symptoms of many skin diseases as well as an important skin manifestation of systemic dermatoses 1. Elimination of gluten from the diet usually leads to resolution of skin symptoms. In pathogenesis, hla dq2 and hla dq8 haplotypes are known to be involved, both in dermatitis herpetiformis and in celiac disease 7,8.
Pdf the diagnosis and treatment of dermatitis herpetiformis. Dermatitis herpetiformis dh is an autoimmune disease closely related to celiac disease cd. While the disease is not uncommon among adolescents, dh is rarely seen in prepubertal patients. Dermatitis herpetiformis dh is an uncommon autoimmune cutaneous eruption associated with gluten sensitivity. Linking gluten sensitivity to dh led to the adoption of the glutenfree diet as a key component of treatment. Dermatitis herpetiformis is a disease of the skin caused by the deposition of iga in the papillary dermis, which triggers an immunologic cascade, resulting in neutrophil recruitment and complement activation. Dermatitis herpetiformis dh is an autoimmune blistering disorder associated with a glutensensitive enteropathy gse. The aim of this research was to assess the role of il31 in development of itch as well as to measure its intensity. Guidelines for the diagnosis and treatment of dermatitis. There are granular deposits at the dermal papillary tips with fewer deposits beneath epidermal rete ridges a, reduced from x 96, b, x 195. Guidelines for the diagnosis and treatment of dermatitis herpetiformis.
Recent studies have demonstrated that iga and antibodies against epidermal transglutaminase 3 play an important role in the pathogenesis of dermatitis herpetiformis. Pathophysiology, clinical presentation, diagnosis and treatment. Dermatitis herpetiformis is the result of an immunologic response to chronic stimulation of the gut mucosa by dietary gluten. With dermatitis, your skin will typically look dry, swollen, and red. Keywords autoimmune bullous diseases, celiac disease, tissue transglutaminase, antiendomysium antibodies, antitissue transglutaminase antibodies, gluten sensitivity, dapsone. The presence of iga is considered essential in the pathogenesis of dermatitis herpetiformis. Request pdf pathophysiology of dermatitis herpetiformis.
Over the last two decades a rapid expansion of our knowledge regarding dermatitis herpetiformis has occurred, including the discovery of iga in the skin, the discovery of an associated glutensensitive enteropathy, the noting of an increased prevalence. The aim of this study was to investigate whether apoptosis may contribute to the pathogenesis of epidermal changes in dermatitis herpetiformis dh and, in particular, whether certain apoptosisrelated markers such as bax, bcl2, fas and fas ligand fasl take part in this process. Tissue transglutaminase is the major autoantigen in cd, while epidermal transglutaminase is most closely linked to dh. Dermatitis herpetiformis was first described by louis adolphus duhring in 1884 at the university of pennsylvania. The classic location for dermatitis herpetiformis lesions is on the extensor surfaces of the elbows, knees. The role of intereukin31 in pathogenesis of itch and its intensity in a course of bullous pemphigoid and dermatitis herpetiformis liliannakulczyckasiennicka,annacynkier,elhbietawaszczykowska. It is a blistering skin disorder that manifests clinically with a chronic and intensely pruritic rash with a symmetrical distribution. The role of intereukin31 in pathogenesis of itch and its. Dermatitis herpetiformis dh was initially described by louis duhring in 1884. Previously, pemphigus was described using various terms, including herpetiform pemphigus, acantholytic herpetiform dermatitis, pemphigus controlled by sulfapyridine, and mi.
A possible immunological mechanism for the pathogenesis of dermatitis herpetiformis with reference to coeliac disease. Dermatitis herpetiformis dh is a chronic pruritic cutaneous eruption associated with glutensensitive enteropathy celiac disease cd and immunoglobulin a iga deposition in the skin. Genetic background, gluten consumption, and abnormal immune and autoimmune reactions are the most important pathogenetic factors, but other agents also participate in. Epidemiology, pathogenesis, and clinical presentation dermatitis herpetiformis dh is an autoimmune disease that is linked to gluten sensitivity. Dermatology and metabolism branches, national cancer institute, national institutes of health bethesda maryland u. Depending on the type of dermatitis you have, causes vary. Salmi1,2 1celiac disease research center, faculty of medicine and health technology, tampere university, tampere, finland. Dermatitis herpetiformis dh is a cutaneous manifestation of gluten intolerance characterized by an intensely pruritic, papulovesicular eruption with a. Dermatitis herpetiformis dh, also known as duhringbrocq dermatitis, is a chronic, recurrent disease, secondary to gluten hypersensitivity which main clinical manifestation is the occurrence of a papulovesicular pruriginous rash. Response of the skin in dermatitis herpetiformis to a gluten free diet, with reference to jejunal morphology. The role of apoptosis in the pathogenesis of dermatitis. Symmetrical lesion distribution in elbows, shoulder, buttock and knees area 1, 5. The classic location for dermatitis herpetiformis lesions is on the extensor surfaces of the elbows, knees, buttocks, and back. These diseases are characterized by itchiness, red skin and a rash.
Dermatitis herpetiformis occurs in 10% of patients with celiac disease that itself affects 1 in 100200 canadians. A strict gluten withdrawal from diet represents the basis for treatment. Disease name and synonyms dermatitis herpetiformis. Dermatitis herpetiformis and sle were diagnosed in a 15yearold girl with. A model for cutaneous manifestations of gastrointestinal inflammation dermatitis herpetiformis dh is an autoimmune blistering skin. Cd, and pathognomonic iga deposits in the skin and oral mucosa. The diagnosis and treatment of dermatitis herpetiformis emiliano antiga, marzia caproni department of surgery and translational medicine. Dermatitis herpetiformis dh is a rare autoimmune disease linked to gluten sensitivity with a chronicrelapsing course. Review article the pathogenesis of dermatitis herpetiformis stephen i.
Pdf dermatitis herpetiformis dh is an inflammatory cutaneous disease with a chronic relapsing course, pruritic polymorphic lesions, and. Cormane, immunofluorescent studies of the skin in lupus erythernatosus and other diseases pathologia europ 2. Both conditions are mediated by the iga class of autoantibodies and the diagnosis of dh is dependent on detection of. Scanning power view of dermatitis herpetiformis shows vesicular reaction pattern figure 1, characterized by foci or small zones of subepidermal separation figures 2 and 3. Pathogenesis of dermatitis herpetiformis the binding site and origin of iga deposits granular iga deposits in dermal papillae are characteristic for dh. Diagnosis dermatitis herpetiformis is only diagnosed and confirmed by a dermatologist obtaining a slight.
Histologically, the blister is situated between th. Dermatitis herpetiformis and celiac disease are both primarily associated with the hladq alpha 10501, beta 102 or the hladq alpha 103, beta 10302 heterodimers. Dermatitis herpetiformis dh is an autoimmune disease that is linked to gluten sensitivity and has a clear relationship to celiac disease. 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. Atopic dermatitis, also known as atopic eczema, is a chronic pruritic skin condition affecting approximately 17. The imbalance between metalloproteinases and their tissue inhibitors is involved in the pathogenesis of dermatitis herpetiformis agnieszka zebrowska 0 3 joanna narbutt 0 3 anna sysajedrzejowska 0 3 jozef kobos 1 3 elzbieta waszczykowska 2 3 0 department of dermatology and venereology, medical university of lodz, 5 krzemieniecka street, 94 017. Review article ced clinical and experimental dermatology cpd dermatitis herpetiformis t. The diagnosis and treatment of dermatitis herpetiformis emiliano antiga, marzia. Dermatitis herpetiformis dh is a chronic skin condition associated with coeliac disease. Pemphigus herpetiformis is a clinical variant of pemphigus that combines the clinical features of dermatitis herpetiformis with the immunopathologic features of pemphigus. The lesions are typically distributed over the elbows, knees, and buttocks. Dermatitis herpetiformis is characterized by grouped excoriations, erythematous, urticarial plaques, and papules with vesicles. Oral findings in dermatitis herpetiformis and coeliac disease. These data have led to the hypothesis that gluten and the enteropathy it incites are integral factors in the pathogenesis of dh.
Epidermal transglutaminase appears to be the dominant autoantigen in dermatitis herpetiformis. Dermatitis herpetiformis presenting as pseudovasculitis. Download article pdf view full text htmlmachine readable. In these patients, vesicles erythematous and bullae on the anterior and posterior trunk had found.
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