Dermatitis herpetiformis dh is a rare autoimmune disease linked to gluten sensitivity with a chronicrelapsing course. Histologically, the blister is situated between th. Dermatitis herpetiformis dh is a blistering skin disease in which the patients have mostly subclinical gluten sensitive enteropathy, i. Dermatitis herpetiformis presenting as pseudovasculitis. The diagnosis and treatment of dermatitis herpetiformis ccid. Dermatitis herpetiformis is characterized by an itchy blistering rash. The aim of this research was to assess the role of il31 in development of itch as well as to measure its intensity. Dermatitis herpetiformis dh is an autoimmune disease closely related to celiac disease cd.
Guidelines for the diagnosis and treatment of dermatitis herpetiformis. 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. Genetic background, gluten consumption, and abnormal immune and autoimmune reactions are the most important pathogenetic factors, but other agents also participate in. Disease name and synonyms dermatitis herpetiformis. Dermatitis herpetiformis dh is a subepidermal bullous disease characterized by chronic. Onset of dermatitis herpetiformis is usually early to middle adult life but can occur in children and later in life. Dermatitis herpetiformis dh is a chronic autoimmune blistering skin condition, characterised by blisters filled with a watery fluid that is intensely itchy. Linking gluten sensitivity to dh led to the adoption of the glutenfree diet as a key component of treatment. 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. The pathogenesis of dermatitis herpetiformis sciencedirect.
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. The classic location for dermatitis herpetiformis lesions is on the extensor surfaces of the elbows, knees. Tissue transglutaminase is the major autoantigen in cd, while epidermal transglutaminase is most closely linked to dh. 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. What is the pathophysiology of dermatitis herpetiformis. The pathophysiology of dh likely involves a complex interplay between autoimmune factors, such as human leukocyte antigen hla predisposition, genetics, and environment. She was seen in shengzhan by traditional chinese and western doctors. Strober, dermatology and metabolism branches, national cancer institute, national institutes of health, bethesda, maryland, u. 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. The imbalance between metalloproteinases and their tissue.
Dermatitis herpetiformis dh is a cutaneous manifestation of gluten intolerance characterized by an intensely pruritic, papulovesicular eruption with a. Dermatology and metabolism branches, national cancer institute, national institutes of health bethesda maryland u. Review article ced clinical and experimental dermatology cpd dermatitis herpetiformis t. Keywords autoimmune bullous diseases, celiac disease, tissue. Marcos vinicius clarindo, adriana tomazzoni possebon. Pdf dermatitis herpetiformis dh is an inflammatory cutaneous disease with a chronic relapsing course, pruritic polymorphic lesions, and. Like celiac disease, dermatitis herpetiformis is more common in genetically predisposed individuals carrying either the. 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. 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. Lawleyeffect of sulfones on complement deposition in dermatitis herpetiformis and on. Oral findings in dermatitis herpetiformis and coeliac disease. Epidermal transglutaminase appears to be the dominant autoantigen in dermatitis herpetiformis. Atopic dermatitis, also known as atopic eczema, is a chronic pruritic skin condition affecting approximately 17. Review article the pathogenesis of dermatitis herpetiformis stephen i.
The role of intereukin31 in pathogenesis of itch and its. In these patients, vesicles erythematous and bullae on the anterior and posterior trunk had found. Dermatitis herpetiformis is the result of an immunologic response to chronic stimulation of the gut mucosa by dietary gluten. Recent studies have demonstrated that iga and antibodies against epidermal transglutaminase 3 play an important role in the pathogenesis of dermatitis herpetiformis. Both are mediated by iga autoantibodies, triggered by the ingestion of gluten. Dermatitis herpetiformis dh is an uncommon autoimmune cutaneous eruption associated with gluten sensitivity. Pathophysiology, clinical presentation, diagnosis and treatment. Systemic lupus erythematosus and dermatitis herpetiformis. This study aimed to investigate the safety and longterm quality of life and health effects of oat consumption in 312 longterm treated dh patients. Pemphigus herpetiformis is a clinical variant of pemphigus that combines the clinical features of dermatitis herpetiformis with the immunopathologic features of pemphigus. 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.
The classic location for dermatitis herpetiformis lesions is on the extensor surfaces of the elbows, knees, buttocks, and back. Dermatitis herpetiformis dh is a chronic skin condition associated with coeliac disease. The lesions are typically distributed over the elbows, knees, and buttocks. 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. Previously, pemphigus was described using various terms, including herpetiform pemphigus, acantholytic herpetiform dermatitis, pemphigus controlled by sulfapyridine, and mi. Attempts to address the pathophysiology of dermatitis herpetiformis must take the following characteristics into account. Dermatitis herpetiformis was first described by louis adolphus duhring in 1884 at the university of pennsylvania. There was no history of photosensitivity or any other aggravating factors. Keywords autoimmune bullous diseases, celiac disease, tissue transglutaminase, antiendomysium antibodies, antitissue transglutaminase antibodies, gluten sensitivity, dapsone. Cd, and pathognomonic iga deposits in the skin and oral mucosa. Dermatitis herpetiformis dh is a chronic pruritic cutaneous eruption associated with glutensensitive enteropathy celiac disease cd and immunoglobulin a iga deposition in the skin. These diseases are characterized by itchiness, red skin and a rash.
The diagnosis and treatment of dermatitis herpetiformis emiliano antiga, marzia. Pathogenesis of dermatitis herpetiformis the binding site and origin of iga deposits granular iga deposits in dermal papillae are characteristic for dh. A possible immunological mechanism for the pathogenesis of dermatitis herpetiformis with reference to coeliac disease. Guidelines for the diagnosis and treatment of dermatitis. Dermatitis herpetiformis is characterized by grouped excoriations, erythematous, urticarial plaques, and papules with vesicles. Introduction pruritus is one of the main symptoms of many skin diseases as well as an important skin manifestation of systemic dermatoses 1. Epidemiology, pathogenesis, and clinical presentation dermatitis herpetiformis dh is an autoimmune disease that is linked to gluten sensitivity. Elimination of gluten from the diet usually leads to resolution of skin symptoms.
With dermatitis, your skin will typically look dry, swollen, and red. This is a form of nonige cellmediated hypersensitivity related 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. Both conditions are mediated by the iga class of autoantibodies and the diagnosis of dh is dependent on detection of. Affected patients typically develop intensely pruritic inflammatory papules and vesicles on the forearms, knees, scalp, or buttocks picture 1ag.
The diagnosis and treatment of dermatitis herpetiformis emiliano antiga, marzia caproni department of surgery and translational medicine. Dermatitis herpetiformis and sle were diagnosed in a 15yearold girl with. What is the role of food allergies in the pathogenesis of. Cormane, immunofluorescent studies of the skin in lupus erythernatosus and other diseases pathologia europ 2. Symmetrical lesion distribution in elbows, shoulder, buttock and knees area 1, 5. Dermatitis herpetiformis dh is an autoimmune disease that is linked to gluten sensitivity and has a clear relationship to celiac disease. Dermatitis, also known as eczema, is a group of diseases that result in inflammation of the skin. The role of apoptosis in the pathogenesis of dermatitis. Hla, play an important role in the pathogenesis of dh 23. 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 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.
Dermatitis herpetiformis and celiac disease are both primarily associated with the hladq alpha 10501, beta 102 or the hladq alpha 103, beta 10302 heterodimers. 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. Dermatitis is a general term for skin inflammation. It is a blistering skin disorder that manifests clinically with a chronic and intensely pruritic rash with a symmetrical distribution. Diagnosis dermatitis herpetiformis is only diagnosed and confirmed by a dermatologist obtaining a slight.
Dermatitis herpetiformis occurs in 10% of patients with celiac disease that itself affects 1 in 100200 canadians. Dermatitis herpetiformis dh is an autoimmune blistering disorder associated with a glutensensitive enteropathy gse. The pathogenesis of dermatitis herpetiformis in the skin is related to the pathogenesis of celiac disease in the gut. Request pdf pathophysiology of dermatitis herpetiformis.
Dermatitis herpetiformis dh was initially described by louis duhring in 1884. While the disease is not uncommon among adolescents, dh is rarely seen in prepubertal patients. In pathogenesis, hla dq2 and hla dq8 haplotypes are known to be involved, both in dermatitis herpetiformis and in celiac disease 7,8. Salmi1,2 1celiac disease research center, faculty of medicine and health technology, tampere university, tampere, finland.
An evaluation of the diagnostic criteria br j dermatol 90. A model for cutaneous manifestations of gastrointestinal inflammation dermatitis herpetiformis dh is an autoimmune blistering skin. Depending on the type of dermatitis you have, causes vary. Dermatitis herpetiformis prevalence varies across countries. The role of intereukin31 in pathogenesis of itch and its intensity in a course of bullous pemphigoid and dermatitis herpetiformis liliannakulczyckasiennicka,annacynkier,elhbietawaszczykowska. These data have led to the hypothesis that gluten and the enteropathy it incites are integral factors in the pathogenesis of dh. Pdf the diagnosis and treatment of dermatitis herpetiformis. There are granular deposits at the dermal papillary tips with fewer deposits beneath epidermal rete ridges a, reduced from x 96, b, x 195. Response of the skin in dermatitis herpetiformis to a gluten free diet, with reference to jejunal morphology. Download article pdf view full text htmlmachine readable. The presence of iga is considered essential in the pathogenesis of dermatitis herpetiformis. 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.
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