Guidelines for the diagnosis and treatment of dermatitis herpetiformis

Dermatitis herpetiformis is a rare disease that should be considered the cutaneous expression of a gluten-sensitive enteropathy indistinguishable from celiac disease. Dermatitis herpetiformis is often misdiagnosed and to date no guidelines for the management of dermatitis herpetiformis have been published in Literature. The present guidelines have been prepared for dermatologists by the Group for Cutaneous Immunopathology of the Italian Society of Dermatology and Venereology. They reflect the best data available at the time of preparation and the clinical experience of the authors and the members of the Italian Group for Cutaneous Immunopathology. The diagnosis of dermatitis herpetiformis is established clinically, histologically, immunopathologically and serologically. A gluten-free diet (GFD) is the treatment of choice for patients with dermatitis herpetiformis. Dapsone and/or other drugs should be used during the period until the GFD is effective. In conclusion, the present guidelines provide evidence-based guidance for the diagnosis and treatment of dermatitis herpetiformis.

Conflicts of interest

None declared.     

中国特应性皮炎诊疗指南（2020版）解读

 特应性皮炎是一种常见的慢性、复发性、炎症性、瘙痒性皮肤病,严重影响患者生活质量。近年来国内外AD研究进展迅速,为了进一步规范和指导AD的诊断和治疗,中华医学会皮肤性病学分会免疫学组和特应性皮炎协作研究中心组织相关专家对原指南进行了修订。本文就2020年版《中国特应性皮炎诊疗指南》中更新部分作一简要解读。     

Sulfasalazine and dermatitis herpetiformis

Dermatitis herpetiformis that is unable to be controlled using dapsone and a gluten-free diet presents a therapeutic challenge. Three cases that responded well to sulfasalazine are presented. Two cases, who were unable to tolerate dapsone, had a rapid response to sulfasalazine, without apparent side-effects. The third case with dapsone-responsive blistering dermatoses, presumed to be dermatitis herpetiformis on the basis of serology, showed an excellent clinical response to sulfasalazine, but after 6 weeks of therapy had to cease it because of side-effects. Sulfasalazine is metabolized variably to sulfapyridine, a sulphonamide known to be an effective therapy for dermatitis herpetiformis but no longer available. Sulfasalazine should be considered as a management option for dermatitis herpetiformis.     

German S1 guidelines for the diagnosis and treatment of perianal dermatitis (anal eczema)

Perianal dermatitis (anal eczema, perianal eczema) is one of the most common proctological conditions. It may occur as a sequela or a presenting symptom of various proctological, dermatological, allergic or pathogen‐induced disorders. The three main types of anal eczema are irritant‐toxic, atopic and allergic contact dermatitis. Adequate and successful treatment requires a comprehensive diagnostic workup to determine disease etiology and includes treatment/elimination of causative factors as well as nonpharmacological interventions (avoidance of aggravating factors). In addition, adjuvant topical anti‐inflammatory and/or specific symptomatic treatment may be required. The present guidelines contain recommendations for the diagnostic and therapeutic management of perianal dermatitis. Target users of these guidelines are clinicians in the fields of dermatology and proctology, as well as all other specialties involved in the management of patients with perianal dermatitis, both in hospital and office‐based settings.     

Immunoglobulins in the skin in dermatitis herpetiformis and their relevance in diagnosis

Eighty skin biopsies from fifty patients with dermatitis herpetiformis (DH) have been examined for immunoglobulin deposits by direct immunofluorescence. IgA was found in all fifty patients. However, in two patients no IgA was detected in their first biopsy, and it is stressed that if the clinical suspicion of DH is high and no IgA is found in a single biopsy, then the biopsy should be repeated. There are two distinct patterns of immunoglobulin deposition in DH. The most common form of deposition is seen in the dermal papillae, termed the 'papillary' pattern. This pattern was the only one present in sixty-seven of the seventy-eight biopsies. A less common pattern is that of a 'continuous' line along the dermo-epidermal junction. This was the only pattern of immunoglobulin deposition in nine of the seventy-eight biopsies. In two biopsies both the papillary and continuous patterns were present. IgA was found in all seventy-eight of the positive biopsies and was the only immunoglobulin detected in sixty-seven biopsies. In addition to IgA, IgM was present in seven biopsies, and IgG in two biopsies. In one biopsy IgM and IgG were present with the IgA. The detection of IgA in the uninvolved skin in patients with DH is a simple test to perform, and at the present time is the most reliable way of establishing the diagnosis     

Management of dermatitis herpetiformis

The major treatment strategies for DH are gluten restriction or medical treatment with sulfones. Control of the cutaneous manifestations, but not the gastrointestinal changes, is rapid with dapsone. In addition to control of the cutaneous signs and symptoms of DH, dietary gluten restriction also induces improvement of gastrointestinal morphology and is possibly protective against the development of lymphoma.     

疱疹样皮炎研究进展

疱疹样皮炎(dermatitis herpetiformis,DH)是一种与乳糜泻密切相关的自身免疫性大疱性皮肤病,多见于高加索人群,亚洲人群少见.遗传学研究发现高加索人DH的遗传风险因子为HLA-DQ2和HLA-DQ8,已纳入诊断标准,国人DH风险因子为HLA-B?0801和HLA-DRB1?0301,尚未纳入诊断标...     

Atopic disease and dermatitis herpetiformis

Patients with dermatitis herpetiformis were found to have an increased incidence of atopic disorders compared to an age and sex matched control group. The increase in incidence was statistically significant for atopic eczema and any atopic disorder. Serum immunoglobulins (including IgE) were estimated in forty-five dermatitis herpetiformis patients and no significant abnormalities were found.     

Clinical practice guidelines for the management of atopic dermatitis 2018

Atopic dermatitis (AD) is a disease characterized by relapsing eczema with pruritus as a primary lesion. The current strategies to treat AD in Japan from the perspective of evidence‐based medicine consist of three primary measures: (i) the use of topical corticosteroids and tacrolimus ointment as the main treatment for the inflammation; (ii) topical application of emollients to treat the cutaneous barrier dysfunction; and (iii) avoidance of apparent exacerbating factors, psychological counseling and advice about daily life. The guidelines present recommendations to review clinical research articles, evaluate the balance between the advantages and disadvantages of medical activities, and optimize medical activity‐related patient outcomes with respect to several important points requiring decision‐making in clinical practice.