Dermatitis herpetiformis presenting as pseudovasculitis

Kern, Malan; Kim, Kevin H.; Johnson, Gina; Hawkins, Spencer; Wong, Henry K.

doi:10.1016/j.jdcr.2017.06.033

Cited by 4 publications

(3 citation statements)

References 8 publications

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“…Moreover, Naylor et al reported a case of DH presenting with a diffuse petechial rush and microscopic changes consistent with both DH and vasculitis (56). Kern et al reported a patient with DH presenting as pseudovasculitis, characterized by a diffuse petechial rush and a large ulcer at his extensor forearm, and histopathologic and direct immunofluorescence (DIF) findings consistent with DH without additional signs of small-vessel inflammation (57). A possible hypothesis of the association between DH and vascular damage might be the presence of perivascular IgA immune complexes leading to small vessel inflammation.…”

Section: The Epidemiology Of Dermatitis Herpetiformis: a (Rare) Diseamentioning

confidence: 99%

Dermatitis Herpetiformis: Novel Perspectives

et al. 2019

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Dermatitis herpetiformis (DH) is an inflammatory disease of the skin, considered the specific cutaneous manifestation of celiac disease (CD). Both DH and CD occur in gluten-sensitive individuals, share the same Human Leukocyte Antigen (HLA) haplotypes (DQ2 and DQ8), and improve following the administration of a gluten-free diet. Moreover, almost all DH patients show typical CD alterations at the small bowel biopsy, ranging from villous atrophy to augmented presence of intraepithelial lymphocytes, as well as the generation of circulating autoantibodies against tissue transglutaminase (tTG). Clinically, DH presents with polymorphic lesions, including papules, vesicles, and small blisters, symmetrically distributed in typical anatomical sites including the extensor aspects of the limbs, the elbows, the sacral regions, and the buttocks. Intense pruritus is almost the rule. However, many atypical presentations of DH have also been reported. Moreover, recent evidence suggested that DH is changing. Firstly, some studies reported a reduced incidence of DH, probably due to early recognition of CD, so that there is not enough time for DH to develop. Moreover, data from Japanese literature highlighted the absence of intestinal involvement as well as of the typical serological markers of CD (i.e., anti-tTG antibodies) in Japanese patients with DH. Similar cases may also occur in Caucasian patients, complicating DH diagnosis. The latter relies on the combination of clinical, histopathologic, and immunopathologic findings. Detecting granular IgA deposits at the dermal-epidermal junction by direct immunofluorescence (DIF) from perilesional skin represents the most specific diagnostic tool. Further, assessing serum titers of autoantibodies against epidermal transglutaminase (eTG), the supposed autoantigen of DH, may also serve as a clue for the diagnosis. However, a study from our group has recently demonstrated that granular IgA deposits may also occur in celiac patients with non-DH inflammatory skin diseases, raising questions about the effective role of eTG IgA autoantibodies in DH and suggesting the need of revising diagnostic criteria, conceivably emphasizing clinical aspects of the disease along with DIF. DH usually responds to the gluten-free diet. Topical clobetasol ointment or dapsone may be also applied to favor rapid disease control. Our review will focus on novel pathogenic insights, controversies, and management aspects of DH.

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Section: The Epidemiology Of Dermatitis Herpetiformis: a (Rare) Diseamentioning

confidence: 99%

Dermatitis Herpetiformis: Novel Perspectives

et al. 2019

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“…Naylor et al ( 121 ) published a DH patient presented with diffuse petechial rush and microscopic changes consistent with both DH and vasculitis. Kern et al ( 122 ) described a case of DH presenting as pseudovasculitis with diffuse petechial rush and an ulcer on forearm, and histopathology and DIF were both compatible with DH, with no signs of vasculitis. Finally, atypical clinical presentation of DH includes palmoplantar keratosis ( 123 ), wheals of chronic urticaria ( 124 ) and lesions mimicking prurigo pigmentosa ( 125 ).…”

Section: Dermatitis Herpetiformis: Typical and Atypical Manifestationsmentioning

confidence: 99%

Skin gluten-related disorders: new and old cutaneous manifestations to be considered

et al. 2023

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The term gluten-related disorders (GRD) refer to a spectrum of different clinical manifestations triggered by the ingestion of gluten in genetically susceptible individuals, including coeliac disease (CD), wheat allergy and non-celiac gluten sensitivity (NCGS). GRD are characterized by a large variety of clinical presentations with both intestinal and extra-intestinal manifestations. The latter may affect almost every organ of the body, including the skin. Besides the well-known association between CD and dermatitis herpetiformis, considered as the cutaneous specific manifestation of CD, many other muco-cutaneous disorders have been associated to GRD. In this review, we analyzed the main features of dermatological diseases with a proven association with GRD and those that improve after a gluten-free diet, focusing on the newly described cutaneous manifestations associated with NCGS. Our main hypothesis is that a “cutaneous-gluten sensitivity,” as specific cutaneous manifestation of NCGS, may exist and could represent a diagnostic marker of NCGS.

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“…Кроме того, представлены данные о пациенте с герпетиформным дерматитом, являющимся по сути псевдоваскулитом, характеризующимся диффузным петехиальным приливом и большой язвой на разгибательной поверхности предплечья. Возможной гипотезой о связи между герпетиформным дерматитом и повреждением сосудов может быть наличие периваскулярных иммунных комплексов IgA, приводящих к воспалению мелких сосудов [32].…”

Section: клиническая картина заболеванияunclassified

Dermatitis herpetiformis Duhring as one of the forms of gluten-associated pathology: a review of the literature and a description of a clinical case

Klimov¹,

Kuryaninova

Dmitrieva

et al. 2022

Medicinskij sovet

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This review presents information on the prevalence, pathogenesis, clinical manifestations, diagnosis and treatment of Duhring’s dermatitis herpetiformis. Although the disease was first clinically described in 1884 by the American dermatologist L.A. Duhring, the study of its pathogenesis and the search for prognostic markers of its occurrence continue. Against the background of a significant expansion of ideas about gluten-dependent diseases and conditions, views on the mechanisms of autoimmune skin damage in dermatitis herpetiformis are detailed. A strong association with hereditary predisposition through the major human leukocyte histocompatibility complex (HLA) DQ2 and DQ8, and a role of epidermal transglutaminase as a major autoantigen in dermatitis herpetiformis are shown. The hypotheses explaining the decline in the incidence of dermatitis herpetiformis in recent decades against the background of increased and more effective serological screening and the resulting earlier diagnosis of celiac disease are commented on. A typical clinical picture of dermatitis herpetiformis, in which erythematous papules, plaques, vesicles are seen, usually clustered on the flexural surfaces of the extremities. Secondary elements are erosions, excoriations and crusts due to rupture of blisters and due to scratching caused by intense itching. A generally favourable prognosis for life and disease is shown with a gluten-free diet and the use of dapsone, glucocorticoids and, if these are ineffective, immunosuppressants. The authors describe a clinical case of the disease in an adolescent girl with a typical clinical history and characteristic rashes on the extensor surfaces of the limbs. The authors show that drug therapy without a gluten-free diet cannot be considered effective, and that the diet for dermatitis herpetiformis, like that for celiac disease, is lifelong. The growing understanding of gluten-associated pathology, which includes dermatitis herpetiformis, in recent decades has led to an intensive search for diagnostic and prognostic markers, as well as the development of ways to correct this group of diseases, including those not related to the lifelong elimination of cereal prolamines.

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Dermatitis herpetiformis presenting as pseudovasculitis

Cited by 4 publications

References 8 publications

Dermatitis Herpetiformis: Novel Perspectives

Dermatitis Herpetiformis: Novel Perspectives

Skin gluten-related disorders: new and old cutaneous manifestations to be considered

Dermatitis herpetiformis Duhring as one of the forms of gluten-associated pathology: a review of the literature and a description of a clinical case

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