[Hygiene care and local treatment of skin and mucous membranes in autoimmune bullous diseases]. / Soins d'hygiène et traitements locaux de la peau et des muqueuses dans les maladies bulleuses auto-immunes.

As part of its therapeutic patient education program, the Avicenne hospital in the Paris region invites patients with an autoimmune bullous disease to a workshop dedicated to local skin and mucous membrane care. Together, a nurse, patient partners and patients review best practices in hygiene care and treatment of the skin, eyes, nose, mouth, genitals and anus. This is essential for healing lesions and avoiding local complications.

[What's new in research?] / Quoi de neuf en recherche?

A traditional lecture given during the annual meeting of the French Society of Dermatology in Paris summarizes the highlights of the scientific literature over the past year. In the current article the selection of the 2017-2018 period retains the following areas of interest: role of microbiome in the response to anti-PD-1 and in autoimmunity, PI3Kδ inhibitors in autoimmune bullous diseases, diagnostic and therapeutic applications of CRISPR/Cas, arrival of CAR-T cells therapy into clinical practice, gene therapy successes, use of targeted therapies in genodermatoses and integration of genetics in primary care. © 2018 Elsevier Masson SAS. All rights reserved.

[Autoimmune bullous diseases with gingival expression: A proposed non-iatrogenic gingival biopsy technique]. / Maladies bulleuses auto-immunes à expression gingivale : proposition d'une technique de biopsie gingivale non iatrogène.

BACKGROUND: Gingival expression of autoimmune bullous diseases (AIBD) may be inaugural, exclusive or dominant (mucous membrane pemphigoid, pemphigus vulgaris). Histology and direct immunofluorescence are essential to diagnosis. The location of the biopsy and the surgical technique determine the histological quality of the tissue sample. However, gingival tissue is often considered fragile and easily impaired during biopsy. We suggest an original biopsy protocol for the gingival papillae that is simple to perform, non-iatrogenic, and readily accessible to all practitioners who usually treat AIBD patients presenting isolated gingival expression (dermatologists, stomatologists, odontology specialists, ENT specialists). PATIENTS AND METHODS: We conducted a retrospective study from 2012 to 2017 identifying all patients presenting AIBD with gingival expression for whom we performed papillary gingival biopsy for diagnostic ends. Our main objective was to determine the diagnostic efficacy and safety of this surgical technique. RESULTS: Over the study period, 34 papillary gingival biopsies were taken from 19 patients : 15 for histopathological examination and 19 for direct immunofluorescence. Of the 34 biopsies, only one could not be properly analyzed due to lack of epithelium and a second tissue sample was therefore necessary. No short- or long-term complications occurred during post-operative follow-up. CONCLUSION: Gingival papilla biopsy is perfectly suited to the histopathological and immunohistochemical examinations needed for diagnosis of AIBD with isolated gingival expression. This surgical technique shows great efficacy and very good safety. However, additional studies are necessary to confirm our preliminary results, in particular the absence of iatrogenic effects.

[Pemphigus and herpes: Multicentre survey and literature review]. / Pemphigus et herpès : enquête multicentrique et revue de la littérature.

BACKGROUND: Although herpes superinfection is a well-known complication of pemphigus, it has not been widely investigated. AIM: To investigate the frequency and features of herpes infection in patients with ongoing pemphigus. PATIENTS AND METHODS: We carried out a multicenter retrospective study between 2008 and 2016 in patients with newly diagnosed pemphigus presenting active herpes infection. Clinical, virological, immunological and therapeutic data were collated. We performed a literature review for pemphigus and herpes. RESULTS: Among the 191 pemphigus patients, screening for herpes (PCR or culture) was carried out in 11 to 71 % of subjects, depending on the center in question. Twenty-four patients (12 women, mean age 58 years) presented at least one episode of herpes infection. The frequency of positivity ranged from 0 to 42 % by center. Twenty-one cases consisted of pemphigus vulgaris and infection occurred at a mucosal site in 19 patients. Herpes infection was identified at the time of diagnosis in 15 patients and 17 patients received no specific treatment for their pemphigus. The virus was identified using PCR in 23 cases. Ten patients subsequently received prophylactic treatment for herpes. The mean duration of follow-up was 36 months (0-89 months). Thirteen of the 24 patients had 23 relapses of pemphigus; PCR testing for herpes was performed 19 times and was positive in 6 cases (31.5 %). CONCLUSION: Our study showed wide variation in the incidence of herpes superinfection in patients with pemphigus, reflecting the different screening approach at each center (being performed either routinely or only in the event of strong suspicion). The prognostic value of routine screening for herpes in patients with active pemphigus lesions remains to be demonstrated by further prospective investigations.

[Oral care recommendations for patients with oral autoimmune bullous diseases]. / Recommandations pour la prise en charge buccodentaire des patients atteints de maladies bulleuses auto-immunes avec atteinte buccale.

BACKGROUND: Autoimmune bullous diseases (AIBD) may cause chronic oral lesions that progress insidiously. AIMS: To provide recommendations for optimal oral-dental management of patients presenting AIBD with oral involvement. PATIENTS AND METHODS: In the absence of scientific studies with high levels of proof, these recommendations have been drawn up at two meetings by a committee of experts on AIBD comprising 7 dermatologists, 1 stomatologist, 1 maxillofacial surgeon, 2 odontologists and 4 parodontologists. RESULTS: The oral lesions associated with AIBD may be classified into three grades of severity: severe (generalised erosive gingivitis affecting at least 30% of dental sites), moderate (localised erosive gingivitis affecting less than 30% of dental sites) and controlled (no erosive oral lesions). Good oral-dental hygiene suited to the severity of the oral lesions, must be practised continually by these patients so as to avoid the formation of dental plaque, which aggravates symptoms. Dental and parodontal care must be considered in accordance with the severity grade of the oral lesions: in severe cases, the dental plaque must be eliminated manually with a curette, but several types of care (descaling, treatment for tooth decay, non-urgent extractions, etc.) must be suspended until the grade of severity is moderate or until the disease is stabilised.

[Autoimmune blistering diseases]. / Les maladies bulleuses auto-immunes.

Autoimmune blistering diseases are characterized by the production of pathogenic autoantibodies that are responsible for the formation of epidermal blisters. Major advances in the understanding of the pathogenesis of these disorders have allowed the development of new therapeutic agents. Recent epidemiologic data showed that bullous pemphigoid mainly affects elderly patients. Bullous pemphigoid is often associated with degenerative neurologic disorders. A major increase in the incidence of bullous pemphigoid has been observed in France. Treatment of bullous pemphigoid is mainly based on superpotent topical corticosteroids. The role of desmosomal proteins has been demonstrated in the initiation, propagation and persistence of the autoimmune response in pemphigus. Several studies have shown a correlation between anti-desmoglein antibody titers and disease activity. Pemphigus susceptibility genes have been identified. Oral corticosteroids remain the mainstay of pemphigus treatment. Dramatic and long-lasting improvement has been recently obtained with rituximab in recalcitrant types of pemphigus. Other autoimmune junctional blistering diseases are rare entities, whose prognosis can be severe. Their diagnosis has been improved by the use of new immunological assays and immunoelectronic microscopy. Immunosupressants are widely used in severe types in order to prevent mucosal sequelae.