An update on the cutaneous manifestations of coeliac disease and non-coeliac gluten sensitivity

Graziano, Mario; Rossi, Mauro

doi:10.1080/08830185.2018.1533008

Cited by 17 publications

(29 citation statements)

References 91 publications

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“…DH is a rare disease that occurs prevalently in Caucasian individuals. In Europe and USA, the prevalence of DH ranges from 11.2 to 75.3 per 100.000, with the highest reported in Finland; whereas, the incidence ranges from 0.4 to 2.6 per 100.000 people per year (14–16). DH is extremely rare among African and Asian populations.…”

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

confidence: 99%

“…DH can occur at any age, but typically occurs during adulthood, and mostly between the third and the fourth decade of life (14). Interestingly, in one Finnish study including 477 patients diagnosed with DH over a 40 year period, the patients' age at DH diagnosis was shown to significantly increase over time.…”

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

confidence: 99%

“…Although DH is the specific cutaneous manifestation of CD, several other skin diseases secondary to gluten ingestion are increasingly reported, especially in the last years, when the focus on gluten intolerance has grown steadily. In fact, despite the exclusion of gluten from the diet is even becoming a fashion, data from the literature demonstrate a higher incidence of skin diseases in patients with gluten-related disorders (14).…”

Section: Gluten and The Skin: Not Always Dermatitis Herpetiformismentioning

confidence: 99%

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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%

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

confidence: 99%

Section: Gluten and The Skin: Not Always Dermatitis Herpetiformismentioning

confidence: 99%

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Dermatitis Herpetiformis: Novel Perspectives

et al. 2019

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“…Dermatitis herpetiformis (DH) is considered an extraintestinal manifestation of CD. In children it is relatively rare (in Finland only 4% of all DH cases are children) ( 79 ), but in some series from other countries it seems to be more frequent in the pediatric age ( 80 ). It is particularly interesting to note that, in contrast to CD, the annual incidence rate is decreasing.…”

Section: Clinical Manifestationsmentioning

confidence: 99%

“…It has been hypothesized that subclinical CD may predispose to DH, but what renders some individuals more prone to the development of skin lesions is unknown. The disease starts in the gut and evolves with the deposition in the papillary dermis of immune complexes of TG3 and high avidity anti-TG3 IgA antibodies ( 79 ).…”

Section: Clinical Manifestationsmentioning

confidence: 99%

Extra-Intestinal Manifestations of Coeliac Disease in Children: Clinical Features and Mechanisms

et al. 2019

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Celiac disease (CD) is a systemic autoimmune disease due to a dysregulated mucosal immune response to gluten and related prolamines in genetically predisposed individuals. It is a common disorder affecting ~1% of the general population, its incidence is steadily increasing. Changes in the clinical presentation have become evident since the 80s with the recognition of extra-intestinal symptoms like short stature, iron deficiency anemia, altered bone metabolism, elevation of liver enzymes, neurological problems. Recent studies have shown that the overall prevalence of extra-intestinal manifestations is similar between pediatric and adult population; however, the prevalence of specific manifestations and rate of improvement differ in the two age groups. For instance, clinical response in children occurs much faster than in adults. Moreover, an early diagnosis is decisive for a better prognosis. The pathogenesis of extra-intestinal manifestations has not been fully elucidated yet. Two main mechanisms have been advanced: the first related to the malabsorption consequent to mucosal damage, the latter associated with a sustained autoimmune response. Importantly, since extra-intestinal manifestations dominate the clinical presentation of over half of patients, a careful case-finding strategy, together with a more liberal use of serological tools, is crucial to improve the detection rate of CD.

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