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Erythema multiforme (EM) is a muco-cutaneous inflammatory disease mainly triggered by herpes simplex virus (HSV) recurrences. Association of EM and circulating auto-antibodies against plakins (anti-PLK-Abs [EM-PLK+]) has been reported. However, little is known about this subset of EM.We aimed to describe the clinical and immunological features and response to treatment of EM-PLK+.We conducted a retrospective multicentric study of EM-PLK+ selected from the database of the immunological laboratory of Bichat hospital, Paris, France, from January 2009 to December 2020. Anti-PLK-Abs were detected in ≥1 immunological tests: immunofluorescence assay, immunoblotting and/or ELISA. Patients with alternative diagnoses were excluded.We included 29 patients (16 women, median age 25 [range 2-58] years). EM-PLK+ were mostly major (EM with ≥2 mucosal involvements; n = 24, 83%) and relapsing (≥2 flares; n = 23, 79%). Cutaneous lesions were target (n = 13, 54%) and target-like lesions (n = 9, 38%) with usual topography (acral, n = 19, 79%; limbs, n = 21, 88%). Mucosal lesions affected the mouth (n = 27, 96%) and genitalia (n = 19, 68%), with a median of 2 [range 0-5] mucous membranes. EM-PLK+ were suspected as certain or possible postherpetic (EM-HSV) in 19 cases (65.5%); no triggering factors were detected in 9 (31%) patients. Desmoplakin-I/II Abs were the most frequent anti-PLK-Abs (n = 20, 69%); envoplakin and periplakin Abs were detected in 11 and 9 cases. Relapsing EM-PLK+ (n = 23) were still active (≥1 flare within 6 months) in 13 (57%) patients despite immunosuppressive therapy (n = 8, 62%). Antiviral drugs were ineffective in preventing relapse in 15/16 (94%) EM-HSV.The rationale for anti-PLK-Ab detection in EM is not elucidated. More systematic research of anti-PLK-Abs is warranted to better understand whether this association reflects humoral immune activity in a subset of EM or is fortuitous, related to an epitope spreading process. However, EM-PLK+ seems to be associated with major and relapsing subtypes, and difficult-to-treat cases.

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Background Supportive care is the cornerstone of management of adult and paediatric Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). However, consensus on the modalities of supportive care is lacking.Objectives Our aim in this international multicentric Delphi exercise was to establish a multidisciplinary expert consensus to standardize recommendations regarding supportive care in the acute phase of SJS/TEN.Methods Participants were sent a survey via the online tool SurveyMonkey, consisting of 103 statements organized into 11 topics: multidisciplinary team composition, suspect drug management, infection prevention, fluid resuscitation and prevention of hypothermia, nutritional support, pain and psychological distress management, management of acute respiratory failure, local skincare, ophthalmological management, management of other mucosa, and additional measures. Participants evaluated the level of appropriateness of each statement on a scale of 1 (extremely inappropriate) to 9 (extremely appropriate). The results were analysed according to the RAND/UCLA Appropriateness Method.Results Forty-five participants from 13 countries (on three continents) participated. After the first round, a consensus was obtained for 82.5% of the 103 initially proposed statements. After the second round, a final consensus was obtained for 102 statements.Conclusions We have reached an international Delphi-based consensus on best supportive care practice for SJS/TEN. Our expert consensus should help guide physicians in treating patients with SJS/TEN and thereby improve short-term prognosis and the risk of sequelae.

European guidelines propose a 0·5 mg kgIn a prospective international study, we consecutively included all patients diagnosed with BP. Patients received a 0·5 mg kgIn total, 198 patients were included between 2015 and 2017. The final analysis comprised 190 patients with a mean age of 80·9 (SD 9·1) years. Control of disease activity was achieved at day 21 in 119 patients [62·6%, 95% confidence interval (CI) 55·3-69.5]; 18 of 24 patients (75%, 95% CI 53·3-90·2), 75 of 110 patients (68·8%, 95% CI 59·2-77·3) and 26 of 56 patients (46.4%, 95% CI 33·0-60·3) had mild, moderate and severe BP, respectively (P = 0·0218). A total of 30 patients died during the study. The overall Kaplan-Meier 1-year survival was 82·6% (95% CI 76·3-87·4) corresponding to 90·9%, 83·0% and 80·0% rates in patients with mild, moderate and severe BP, respectively (P = 0·5). Thresholds of 49 points for BPDAI score and 70 points for Karnofsky score yielded maximal Youden index values with respect to disease control at day 21 and 1-year survival, respectively.A 0·5 mg kg

Propylthiouracil (PTU) is a synthetic antithyroid drug that can induce ANCA-associated vasculitis.A 27-year-old woman diagnosed with Graves' disease was on PTU for the past 10 years. She developed purpuric lesions of the legs and on the tip of the nose diagnosed as vasculitis. ANCAs were positive, with anti-MPO and anti-PR3 on blood ELISA. After discontinuation of PTU, she was able to fully recover.All synthetic antithyroid drugs can induce ANCA-associated vasculitis, more often PTU. In most cases, antibodies are directed against MPO. Dual anti-MPO and anti-PR3 positivity is possible, but rare. The mechanism could be through an accumulation of PTU in neutrophils, altering the structure of MPO and making it immunogenic. PTU can also induce ANCA-free or lupus vasculitis, maculopapular rashes or urticaria. Many other drugs can induce ANCA-associated vasculitis.

Hemophagocytic lymphohistiocytosis (HLH) is a rare syndrome frequently secondary to infectious disease, especially in immuno-compromised patients. We report a HLH secondary to disseminated nocardiosis and Streptomyces spp pulmonary infection.A 69-years-old women had recent subcutaneous nodules of the forearms and loins associated with peripheral neuropathy and pulmonary nodule of the right upper lobe. Cutaneous biopsy revealed granuloma. Cutaneous lesions worsened and the patient developed a HLH with probable cardiac and neurological involvement, associated with cutaneous granulomatosis and diffuse polyclonal lymphocyte proliferation. Nocardia PCR was positive in cutaneous biopsy. Pulmonary samples revealed Streptomyces in culture and Nocardia in PCR. The evolution under antibiotic treatment was favorable.Recent diagnosis of HLH without obvious etiology should lead to etiological investigation, including the search for infections with slow-growing bacteria such as Nocardia or Streptomyces spp.

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).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.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.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.

Although herpes superinfection is a well-known complication of pemphigus, it has not been widely investigated.To investigate the frequency and features of herpes infection in patients with ongoing pemphigus.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.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 %).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.