The diagnosis and treatment of dermatitis herpetiformis

Clinical, Cosmetic and Investigational Dermatology Dovepress open access to scientific and medical research Review Clinical, Cosmetic and Investigational Dermatology downloaded from https://www.dovepress.com/ by 213.32.59.119 on 12-Jul-2018 For personal use only. Open Access Full Text Article The diagnosis and treatment of dermatitis herpetiformis This article was published in the following Dove Press journal: Clinical, Cosmetic and Investigational Dermatology 13 May 2015 Number of times this article has been viewed Emiliano Antiga Marzia Caproni Department of Surgery and Translational Medicine, Section of Dermatology, University of Florence, Florence, Italy Introduction Correspondence: Emiliano Antiga Department of Surgery and Translational Medicine, Section of Dermatology, University of Florence, Viale Michelangelo 41, 50125 Florence, Italy Tel +39 055 6939 664 Fax +39 055 6939 598 Email Dermatitis herpetiformis (DH) is an inflammatory cutaneous disease with a chronic relapsing course, pruritic polymorphic lesions, and typical histopathological and immunopathological findings. According to several evidences, DH is considered the specific cutaneous manifestation of celiac disease (CD). In fact, both diseases occur in gluten-sensitive individuals, share the same HLA haplotypes (DQ2 and DQ8), and improve following the administration of a gluten-free diet.1 Moreover, patients with DH show typical CD alterations at the small bowel biopsy (ranging from villous atrophy to augmented presence of intraepithelial lymphocytes [IELs]) almost in all the cases, as well as the generation of circulating autoantibodies to tissue transglutaminase (tTG). DH is predominately a disorder of Caucasians,2 although Japanese cases are increasingly reported.3 The incidence of the disease was found to be 11.5 per 100,000 in Scotland4 and ranging from 19.6 to 39.2 per 100,000 in Sweden.5 In a recent study from Finland, the prevalence of DH was found to be 75.3 per 100,000 (eight times lower than the prevalence of CD in that area), while the annual incidence was found to 3.5 per 100,000 over the period 1980–2009, showing a decrease in the last years.6 257 submit your manuscript | www.dovepress.com Clinical, Cosmetic and Investigational Dermatology 2015:8 257–265 Dovepress © 2015 Antiga and Caproni. This work is published by Dove Medical Press Limited, and licensed under Creative Commons Attribution – Non Commercial (unported, v3.0) License. The full terms of the License are available at http://creativecommons.org/licenses/by-nc/3.0/. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. Permissions beyond the scope of the License are administered by Dove Medical Press Limited. Information on how to request permission may be found at: http://www.dovepress.com/permissions.php http://dx.doi.org/10.2147/CCID.S69127 Powered by TCPDF (www.tcpdf.org) Abstract: Dermatitis herpetiformis (DH) is an inflammatory cutaneous disease with a chronic relapsing course, pruritic polymorphic lesions, and typical histopathological and immunopathological findings. According to several evidences, DH is considered the specific cutaneous manifestation of celiac disease, and the most recent guidelines of celiac disease have stated that, in celiac patients with a proven DH, a duodenal biopsy is unnecessary for the diagnosis. In this review, the most recent data about the diagnosis and the management of DH have been reported and discussed. In particular, in patients with clinical and/or histopathological findings suggestive for DH, the finding of granular IgA deposits along the dermal–epidermal junction or at the papillary tips by direct immunofluorescence (DIF) assay, together with positive results for anti-tissue transglutaminase antibody testing, allows the diagnosis. Thereafter, a gluten-free diet should be started in association with drugs, such as dapsone, that are able to control the skin manifestations during the first phases of the diet. In conclusion, although DH is a rare autoimmune disease with specific immunopathological alterations at the skin level, its importance goes beyond the skin itself and may have a big impact on the general health status and the quality of life of the patients. Keywords: dermatitis herpetiformis, celiac disease, diagnosis, treatment, autoimmune disease, inflammatory cutaneous disease Dovepress Clinical, Cosmetic and Investigational Dermatology downloaded from https://www.dovepress.com/ by 213.32.59.119 on 12-Jul-2018 For personal use only. Antiga and Caproni DH usually presents in the fourth and fifth decades, although individuals of any age can be affected. In a recent study from our group investigating 159 patients with DH, approximately 27% of the patients were below the age of 10, and 36% below the age of 20, showing that, at least in Italy, pediatric DH is more common than expected in other countries.7 In 2009, the guidelines for the management of patients with DH were published by our group.1 However, according to recent literature, several new findings have been reported about the clinical and immunopathological features of DH; moreover, the novel guidelines for the management of CD from the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN) were developed in 2012.8 Therefore, an update on the diagnosis and treatment of DH would be helpful to improve the care of the patients. Accordingly, in the next paragraphs, the clinical and the immunopathological features that can help in the diagnosis of DH are reported. Moreover, the management of the disease, which is based both on a gluten-free diet and on medications that can help control DH in the inflammatory phases, as well as its follow-up are discussed. Clinical features DH usually presents with symmetrical, grouped polymorphic lesions consisting of erythema, urticarial plaques, and papules,2,9–11 involving the extensor surfaces of the knees, elbows, shoulders, buttocks, sacral region, neck, face, and scalp. By contrast, herpetiform vesicles, which reflect the name of the disease, may occur later or are often immediately excoriated, resulting in erosions, crusted papules, or areas of postinflammatory dyschromia, and are usually not seen in the patients. Itching of variable intensity and scratching and burning sensation immediately preceding the development of lesions are common.2,9–11 Together with these manifestations, several atypical presentations have been reported in patients with DH, including purpuric lesions resembling petechiae on hands and feet,12–20 leukocytoclastic vasculitis-like appearance,21 palmo-plantar keratosis,22 wheals of chronic urticaria,23 and lesions mimicking prurigo pigmentosa.24 Interestingly, in some cases patients may show erythema or severe pruritus alone, making the diagnosis challenging.25 Finally, patients with DH may present the clinical manif (...truncated)