Dermatitis Herpetiformis, the Celiac Disease of the Skin!

Kotze, Lorete Maria da Silva

doi:10.1590/s0004-28032013000200041

Cited by 19 publications

(30 citation statements)

References 32 publications

(51 reference statements)

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“…Gastrointestinal symptoms occur in 13-20% of patients; steatorrhea (loose, stinky, and glistering stools) appears most often periodically or continually, and less than 10% of patients have intestinal cramps, stomachache, bloating, or an increase in the abdominal circumference [20,36]. In 1/5-1/3 of patients, the absorption disorders of the following are observed: D-xylose, iron, folic acid and vitamin B 12 , and also fats, lactose, glucose, calcium, and zinc; incidentally -anaemia due to iron insufficiency and osteopenia without an increased firm for bone fractures [1,2,40,41]. Sixteen percent of children suffer from chronic diarrhoea, 10% from iron deficiency anaemia, exceptionallygrowth disorders, and only 5% of children that are on normal diet have normal mucosa of the small intestine [42].…”

Section: Obraz Klinicznymentioning

confidence: 99%

“…Sixteen percent of children suffer from chronic diarrhoea, 10% from iron deficiency anaemia, exceptionallygrowth disorders, and only 5% of children that are on normal diet have normal mucosa of the small intestine [42]. About 5% of CD-patients will develop DH symptoms within their lifetime [40].…”

Section: Obraz Klinicznymentioning

confidence: 99%

“…Objawy żołądkowo-jelitowe stwierdza się u 13-20% chorych, najczęściej jest to okresowo lub stale występująca biegunka tłuszczowa (luźne, cuchnące i połyskujące stolce), a u mniej niż 10% skurcze jelit, bóle brzucha, wzdęcia, zwiększenie obwodu brzucha [20,36]. U 1/5-1/3 pacjentów obserwuje się zaburzenia wchłaniania D-ksylozy, żelaza, kwasu foliowego i witaminy B 12 , a także tłuszczów, laktozy, glukozy, wapnia i cynku, incydentalnie anemię z niedoboru żelaza oraz osteopenię bez zwiększonego ryzyka złamań kości [1,2,40,41]. Wśród dzieci 16% ma przewlekłe biegunki, 10% niedokrwistość z niedoboru żelaza, wyjątkowo zaburzenia wzrostu, a tylko 5% dzieci będących na normalnej diecie ma prawidłową błonę śluzową jelita cienkiego [42].…”

Section: Concomitant Diseasesunclassified

“…Wśród dzieci 16% ma przewlekłe biegunki, 10% niedokrwistość z niedoboru żelaza, wyjątkowo zaburzenia wzrostu, a tylko 5% dzieci będących na normalnej diecie ma prawidłową błonę śluzową jelita cienkiego [42]. U ok. 5% pacjentów z CD w ciągu całego życia wystąpią objawy DH [40].…”

Section: Concomitant Diseasesunclassified

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Clinical picture of dermatitis herpetiformis

Rybak-d'Obyrn¹,

Placek²

2019

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Dermatitis herpetiformis is a rare, chronic, autoimmune blistering disease with hypersensitivity to gluten with the presence of intense pruritus. Symptoms of the disease are symmetrical, polymorphic skin lesions in a typical location. It is thought that dermatitis herpetiformis is a specific skin manifestation of celiac disease, with frequent asymptomatic gluten enteropathy. Both diseases are characterized by the same tissue compatibility antigens, the presence of circulating IgA antibodies against endomysium and transglutaminases, and clinical remission on a glutenfree diet. Non-adherence to this diet predisposes to the development of some tumours, autoimmune and thyroid gland diseases. The diagnosis of dermatitis herpetiformis is based on the findings of the characteristic IgA deposits in the skin papilla in the direct immunofluorescence test. Specific serum antibodies are used as an aid in the diagnosis and for monitoring the compliance with gluten free diet. streszczenieOpryszczkowate zapalenie skóry jest rzadką, przewlekłą, autoimmunologiczną chorobą pęcherzową, przebiegającą z nadwrażliwością na gluten i obecnością intensywnego świądu. Skórną manifestacją jest występowanie symetrycznych, polimorficznych zmian skórnych grupujących się w układzie opryszczkowatym w typowej lokalizacji. Schorzenie to jest specyficzną skórną manifestacją choroby trzewnej, z często utajoną enteropatią glutenową. Obie choroby charakteryzuje związek z tymi samymi antygenami zgodności tkankowej, obecnością krążących przeciwciał IgA przeciwko endomysium mięśni gładkich i transglutaminazom oraz kliniczna remisja pod wpływem diety bezglutenowej. Nieprzestrzeganie diety bezgutenowej predysponuje do rozwoju niektórych chorób nowotworowych, autoimmunologicznych i chorób tarczycy. Rozpoznanie opiera się na stwierdzeniu w badaniu immunofluorescencji bezpośredniej charakterystycznych złogów IgA w brodawkach skóry. Oznaczanie swoistych surowiczych przeciwciał wykorzystuje się pomocniczo w diagnostyce oraz do monitorowania przestrzegania diety.

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Section: Obraz Klinicznymentioning

confidence: 99%

Section: Obraz Klinicznymentioning

confidence: 99%

Section: Concomitant Diseasesunclassified

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Clinical picture of dermatitis herpetiformis

Rybak-d'Obyrn¹,

Placek²

2019

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“…This was verified by lysosome assays on a highly purified caricain preparation (Cornell & Stelmasiak, 2011). Since then, the value of caricain extracts has been demonstrated in a clinical trial on patients with dermatitis herpetiformis (Zebrowska et al, 2014) a disease also involving a susceptibility to gluten, but which manifests itself in the form of a skin rash, blisters and itchiness (Fry, 1992;Kotze, 2013). The present work follows experiments (Buddrick, Cornell, & Small, 2015) in which it was shown that an enriched caricain preparation was able to considerably reduce the amount of immunoactive gliadin in wholemeal bread by allowing it to react in the dough during proofing.…”

Section: Introductionmentioning

confidence: 99%

Application of the rat liver lysosome assay to determining the reduction of toxic gliadin content during breadmaking

Cornell

Stelmasiak²,

Small

et al. 2016

Food Chemistry

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Rituximab Treatment for Recalcitrant Dermatitis Herpetiformis

et al. 2017

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ermatitis herpetiformis (DH) is an autoimmune blistering disease (AIBD) characterized by pruritic papulovesicles mostly on extensor surfaces. Conventional histologic analysis of lesional skin displays neutrophilic collections within the dermal papillae with associated subepidermal clefting. Direct immunofluorescence of nonlesional skin demonstrates granular deposits of IgA in dermal papillae and the basement membrane zone. IgA deposited in the skin is antiepidermal transglutaminase 3 (TG3). IgA serum antibodies are directed against TG3, as well as tissue transglutaminase (TG2), the latter of which is a reflection of the associated celiac disease. 1 Treatment is primarily aimed at maintaining a glutenfree diet (GFD), with initiation of dapsone therapy for refractory disease. In some cases, immune-suppressing agents are required for patients not responding to conventional therapy.We present a patient with a recalcitrant case of DH who was treated with rituximab. The patient developed clinical and serological remission with normalization of both IgA TG2 and TG3 antibody levels. He has remained symptom-free for 18 months following treatment. Report of a CaseA man in his 80s presented with a 5-year history of worsening widespread pruritic rash consisting of coalescent erythematous scaling plaques over the arms, legs, back, and buttocks with intermittent scattered vesicles (Figure 1A and B). His only medications were losartan potassium and carvedilol. Conven-tional histologic analysis demonstrated focal subepidermal blister formation with a neutrophil-predominant inflammatory infiltrate, with scattered eosinophils and lymphocytes. Collections of neutrophils in dermal papillae, papillary dermal microabscesses, were noted (Figure 2A). Immunostaining for B lymphocytes had essentially negative results (Figure 2B). Direct immunofluorescence demonstrated granular deposition of IgA in dermal papillae, diagnostic of DH (Figure 2C). Due to the patient's advanced age of presentation, repeated direct immunofluorescence was independently performed, which again confirmed DH. He denied gastrointestinal symptoms and began a GFD along with dapsone, 50 mg/d, but his pruritic rash persisted. With concern for worsening anemia, dapsone therapy was discontinued. Sulfasalazine treatment was initiated at 3 g/d but also was discontinued due to gastrointestinal symptoms. After stopping sulfasalazine, his disease worsened and he developed debilitating pruritus. The GFD was again recommended, but the patient was unable to remain adherent. He started a tapering course of prednisone from 40 to 10 mg/d along with azathioprine, titrated up to 2.5 mg/kg/d. His skin continued to worsen over the subsequent months. Given the demonstrated efficacy of rituximab in other AIBDs and the failure of conventional therapy in this patient, a trial of rituximab was initiated using 4 weekly doses (375 mg/m 2 ) while continuing prednisone, 10 mg/d, and azathioprine, 2.5 mg/kg/d. Pretreatment laboratory tests were performed, including IgG (771 mg/ dL; to convert to g...

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Dermatitis Herpetiformis, the Celiac Disease of the Skin!

Cited by 19 publications

References 32 publications

Clinical picture of dermatitis herpetiformis

Clinical picture of dermatitis herpetiformis

Application of the rat liver lysosome assay to determining the reduction of toxic gliadin content during breadmaking

Rituximab Treatment for Recalcitrant Dermatitis Herpetiformis

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