Dermatitis herpetiformis

The symptoms range in severity from mild to serious, but they are likely to disappear if gluten ingestion is avoided and appropriate treatment is administered.Dermatitis herpetiformis symptoms are chronic, and they tend to come and go, mostly in short periods of time in response to the amount of gluten ingested.The bullae found in the skin affected by dermatitis herpetiformis are subepidermal and have rounded lateral borders.The jejunal mucosa may show partial villous atrophy, but the changes tend to be milder than in coeliac disease.The main autoantigen of dermatitis herpetiformis is epidermal transglutaminase (eTG), a cytosolic enzyme involved in cell envelope formation during keratinocyte differentiation.[7] Various research studies have pointed out different potential factors that may play a larger or smaller role in the development of dermatitis herpetiformis.These antibodies cross-react with eTG, and IgA/eTG complexes deposit within the papillary dermis to cause the lesions of dermatitis herpetiformis.[7] Gliadin proteins in gluten are absorbed by the gut and enter the lamina propria where they need to be deamidated by tissue transglutaminase (tTG).Thus, the B cell presents the foreign peptide (modified gliadin) but produces antibodies specific for the self-antigen (tTG).Macrophages may be stimulated to secrete IL-8 by the same process as is seen in the gut, causing neutrophils to accumulate at sites of high eTG concentrations in the dermal papillae of the skin.As with ordinary celiac disease, IgA against transglutaminase disappears (often within months) when patients eliminate gluten from their diet.[31] Topical steroid medications are also sometimes used in combination with dapsone and a gluten-free diet to alleviate the itchiness associated with the rash.The risks of developing complications from dermatitis herpetiformis decrease significantly if the affected individuals follow a gluten-free diet.Individuals of Northern European descent are most likely to be affected and estimates of the rates of DH in British and Finnish populations range from 30 in 100,000 to 75 in 100,000 people, respectively.[6] There is a slight male predominance in DH for unknown reasons and it is associated with celiac disease and the haplotypes HLA-DQ2 and, less commonly, HLA-DQ8.A researcher suggested in 1979 that the mysterious skin disease was DH based on these symptoms and this regimen of self-treatment.
Its characteristic rash resembles herpes and is the basis of its clinical name.
Cross-reactivity hypothesis for the onset of dermatitis herpetiformis in people with celiac disease
Micrograph of dermatitis herpetiformis: Subepidermal vesicles, with papillary neutrophil microabcesses, with neutrophil, eosinophil and lymphocytes infi ltrates in the superfi cial dermis. However, the histopathology is unspecific in approximately 35–40% of the cases, [ 22 ] and direct immunofluorescence is needed, showing deposition of IgA in the papillary dermis in a granular or fibrillar pattern. [ 23 ]
SpecialtyDermatologychronicblisterscoeliac diseaseherpes virusherpeshuman leukocyte antigenHLA-DQ2HLA-DQ8gluten sensitivityLouis Adolphus Duhringpapulovesicularpapulesvesiclesabdominal painbloatingfatiguecrustsdermisvascular dilatationcellular infiltrationlymphocyteseosinophilsbullaemicroscopeneutrophilsepidermisatrophyautoantigenstransglutaminasegeneticsantibodiesGliadinglutenlamina propriadendritic cellsendocytosepattern recognition receptorspathogen-associated molecular patternsdanger-associated molecular patterninflammationmemory Bmemory Tceliac diseaseautoantigenCD4+ T cellspMHC-IItype I helper T (Th1) cellsnaive B celllymph nodesB cell receptorT cell receptorplasma cellsClass A antibodiesmacrophagesFc regiondermal papillaeList of human leukocyte antigen alleles associated with cutaneous conditionscontact dermatitisdyshidrosisscabiesbullous pemphigoidlinear IgA bullous dermatosisblood testimmunofluorescencedermatitisfalse negativesgluten-free dietDapsoneantibacterialbacteriahemolytic anemiacolchicinelymecyclinenicotinamidetetracyclinesulfamethoxypyridazinesulfapyridineTopical steroidmedicationautoimmune diseaseinsulin-dependent diabeteslupus erythematosusSjögren's syndromesarcoidosisvitiligoalopecia areatadigestive systemcomplicationsosteoporosisprevalenceincidenceJean-Paul MaratThe Death of MaratPemphigus herpetiformisDiseasesDBMedlinePluseMedicineOrphanetEpidermalCallusSeborrheic keratosisAcrochordonMolluscum contagiosumActinic keratosisSquamous-cell carcinomaBasal-cell carcinomaMerkel-cell carcinomaNevus sebaceousTrichoepitheliomaFrecklesLentigoMelasmaMelanomaPostinflammatory hyperpigmentationDermalEpidermal inclusion cystHemangiomaDermatofibroma (benign fibrous histiocytoma)KeloidLipomaNeurofibromaXanthomaKaposi's sarcomaInfantile digital fibromatosisGranular cell tumorLeiomyomaLymphangioma circumscriptumMyxoid cystRashesAtopic dermatitisSeborrheic dermatitisStasis dermatitisLichen simplex chronicusDarier's diseaseGlucagonoma syndromeLangerhans cell histiocytosisLichen sclerosusPemphigus foliaceusWiskott–Aldrich syndromeZinc deficiencyScalingPsoriasisCorporisCrurisManuumFacieiPityriasis roseaSecondary syphilisMycosis fungoidesSystemic lupus erythematosusPityriasis rubra pilarisParapsoriasisIchthyosisBlisteringHerpes simplexHerpes zosterVaricellaBullous impetigoAcute contact dermatitisPemphigus vulgarisPorphyria cutanea tardaEpidermolysis bullosa simplexPapularInsect bite reactionsLichen planusMiliariaKeratosis pilarisLichen spinulosusTransient acantholytic dermatosisLichen nitidusPityriasis lichenoides et varioliformis acutaPustularAcne vulgarisRosaceaFolliculitisImpetigoCandidiasisGonococcemiaDermatophyteCoccidioidomycosisSubcorneal pustular dermatosisTinea versicolorPityriasis albaTuberous sclerosisIdiopathic guttate hypomelanosisLeprosyHypopigmented mycosis fungoidesErythemaDrug eruptionsViral exanthemsToxic erythemaCellulitisAbscessErythema nodosumCarcinoid syndromeFixed drug eruptionUrticariaMultiformeMigransGyratum repensAnnulare centrifugumAb igneThrombocytopenic purpuraActinic/solar purpuraDisseminated intravascular coagulationVasculitisSclerodermamorpheaGranuloma annulareLichen sclerosis et atrophicusNecrobiosis lipoidicaUlcersTelogen effluviumAndrogenic alopeciaTinea capitisLoose anagen syndromeLichen planopilarisFolliculitis decalvansAcne keloidalis nuchaeOnychomycosisParonychiaIngrown nailMucous membraneAphthous stomatitisOral candidiasisLeukoplakiaMucous membrane pemphigoidCicatricial pemphigoidHerpesvirusCoxsackievirusSyphilisSystemic histoplasmosisVesiculobullous diseaseAcantholysisPemphigusPemphigus vegetansof Hallopeauof NeumannPemphigus erythematosusEndemic pemphigusParaneoplastic pemphigusIgA pemphigusSubcorneal pustularIntraepidermal neutrophilicPemphigoidLocalisedGestational pemphigoidPemphigoid nodularisEpidermolysis bullosa acquisitaChildhoodbullousBullous lupus erythematosusPUVA-induced acrobullous dermatosisWheat allergyOat sensitivityGS idiopathic neuropathiesGSE associated conditionsAnti-gliadin antibodiesAnti-transglutaminase antibodiesHLA-DQGluten immunochemistryGluten challenge testList of people diagnosed with coeliac disease