Search

Background: The nature of the COVID-19 pandemic led to concerns among patients and physicians about the potential impact of immunosuppressive treatments for chronic diseases such as psoriasis on the risk of severe COVID-19., Objectives: To describe treatment modifications and determine the incidence of COVID-19 infection among psoriasis patients during the first wave of the pandemic, and identify the factors associated with these events., Methods: Data from PSOBIOTEQ cohort relating to the first COVID-19 wave in France (March to June, 2020), as well as a patient-centred COVID-19 questionnaire, were used to evaluate the impact of lockdown on changes (discontinuations, delays or reductions) in systemic therapies, and to determine the incidence of COVID-19 cases among these patients. Logistic regression models were used to assess associated factors., Results: Among the 1751 respondents (89.3%), 282 patients (16.9%) changed their systemic treatment for psoriasis, with 46.0% of these changes being initiated by the patients themselves. Patients were more likely to experience psoriasis flare-ups during the first wave if they changed their treatment during this period (58.7% vs 14.4%; P < 0.0001). Changes to systemic therapies were less frequent among patients with cardiovascular diseases (P < 0.001), and those aged ≥ 65 years (P = 0.02). Overall, 45 patients (2.9%) reported having COVID-19, and eight (17.8%) required hospitalization. Risk factors for COVID-19 infection were close contact with a positive case (P < 0.001) and living in a region with a high incidence of COVID-19 (P < 0.001). Factors associated with a lower risk of COVID-19 were avoiding seeing a physician (P = 0.002), systematically wearing a mask during outings (P = 0.011) and being a current smoker (P = 0.046)., Conclusions: Discontinuation of systemic psoriasis treatments during the first COVID-19 wave (16.9%) - mainly decided by patients themselves (46.0%) - was associated with a higher incidence of disease flares (58.7% vs 14.4%). This observation and factors associated with a higher risk of COVID-19 highlight the need to maintain and adapt patient-physician communication during health crises according to patient profiles, with the aim of avoiding unnecessary treatment discontinuations and ensuring that patients are informed about the risk of infection and the importance of complying with hygiene rules., Competing Interests: Disclosure of interest HA, YD, ES, and OC have no conflicts of interest to disclose. EM is a consultant and has received speaker remunerations from Novartis, AbbVie, LEO Pharma, Lilly, Celgene, Amgen, and Janssen-Cilag. MAR is a consultant for Pfizer, LEO Pharma, Janssen, Galderma, AbbVie, Novartis, Pierre Fabre, Merck and BMS. MV has undertaken activities as a paid consultant, adviser or speaker for Janssen, AbbVie, MSD, Pfizer, LEO Pharma, Medac, Boehringer Ingelheim, Novartis and Arrow. MBB offers a consultancy service and is an investigator for AbbVie, Amgen, Celgene, Janssen, LEO Pharma, Lilly, MSD, Novartis and Pfizer. AD has undertaken activities as a paid consultant for Sanofi-Aventis, outside of the submitted work. NB has been an investigator for Pfizer and Novartis. She is currently a consultant for Janssen and has received speaker remuneration from Janssen. PJ is a consultant for Roche, GSK, Lilly, Principia Biopharma, and Sanofi-Aventis. DJ is a consultant for AbbVie, Almirall, Amgen, Biogen, Boehringer Ingelheim, Bristol Myers Squibb, Celgene, Fresenius-Kabi, Janssen-Cilag, LEO Pharma, Lilly, MEDAC, Novartis, Pfizer, Sanofi and UCB. HB has undertaken activities as a paid consultant, adviser or speaker for AbbVie, Almirall, Anaptysbio, Amgen, Aristea Therapeutics, Boehringer Ingelheim, Bristol Myers Squibb, Celgene, Janssen, LEO Pharma, Lilly, Novartis, Pfizer, UCB and Xion Pharmaceuticals. He has also received grant funding from Bristol Myers Squibb, Janssen, LEO Pharma and Pfizer. CP has been an investigator and consultant for AbbVie, Amgen, Boehringer Ingelheim, Bristol Myers Squibb, Celgene, GSK, Janssen, LEO Pharma, Lilly, Novartis, Pierre Fabre, Pfizer and Sanofi. FT is head of the Pharmaco-Epidemiology Centre (Cephépi) of the Assistance Publique – Hôpitaux de Paris and of the Clinical Research Unit of Pitié Salpêtrière Hospital, both of which structures have received unrestricted research funding and grants for the research projects handled and fees for consultant activities from a large number of pharmaceutical companies that have contributed indiscriminately to the salaries of its employees. Florence Tubach is not employed by these structures and has not received any personal remuneration from these companies., (Copyright © 2023. Published by Elsevier Masson SAS.)

Introduction: Super-potent topical corticosteroids (CS) are the mainstay of treatment for bullous pemphigoid. Since super-potent topical CS have systemic effects due to their transcutaneous absorption, we assessed whether super-potent CS were responsible for hydro-saline retention (HSR) in bullous pemphigoid patients., Patients and Methods: From 2015 to 2017, patients with newly-diagnosed bullous pemphigoid treated using clobetasol propionate cream at a starting daily dose of 20 to 40 g were subsequently included in a prospective study. HSR was assessed by longitudinally measuring extracellular water (ECW) volume using bioimpedance analysis (BodyStat QuadScan 4000®) from Day 0 to Day 30 after the initiation of topical CS. Other parameters related to HSR such as weight, blood pressure, natriuresis and proteinuria, were also recorded., Results: Twenty-nine patients (14 men and 15 women) of mean age 81.8 ± 9.3 years were included and analysed. The mean ECW volume decreased from Day 0 to Day 7 (18.1 ± 4.2 vs 16.7 ± 2.7, p = 0.0094) and was maintained from Day 7 to Day 30 (16.8 ± 2.8 vs 17.0 ± 3.4 L; p = 0.8040). Patient weight loss at Day 30 (69.9 ± 13.6 vs 72.5 ± 14.2 kg, p = 0.0085) was closely correlated with the decrease in ECW volume (r = 0.6740, p < 0.0001). No significant changes in natriuresis, 24-hour proteinuria or blood pressure were observed from Day 0 to Day 30., Conclusion: We found no evidence of HSR in bullous pemphigoid patients treated with super-potent topical CS. Conversely, ECW volume decreased from Day 0 to Day 30, which was correlated with patient weight loss., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2022 Elsevier Masson SAS. All rights reserved.)

Background: Atopic dermatitis (AD) and psoriasis (Pso) are highly prevalent chronic inflammatory skin diseases. They share similarities regarding severity and impact on quality of life but display differences regarding risk factors, comorbidities, and pathogenesis., Objective: This study sought to assess the prevalence of AD and Pso among the French population, along with associated comorbidities, and to compare these data with those of the age- and gender-adjusted French population with neither AD nor Pso., Methods: The survey was conducted by a polling institute between September 1 and November 30, 2016, with proportional quota sampling being applied to render the study population representative of the French population. In all, 20 012 individuals were selected from among 900,000 internet users aged≥15years., Results: Overall, 20,012 adults (48.8% men; 51.2% women) completed a digital questionnaire. The prevalence of AD was 4.65% [95% confidence interval (CI) 4.36%-4.94%] and that of Pso was 4.42% [95% CI: 4.14%-4.71%]. More AD patients presented≥1 comorbidity compared to subjects without AD (57.04% vs. 49.2%, P<0.0001) and more Pso patients presented≥1 comorbidity compared to subjects without Pso (60.68% vs. 49.05%, P<0.0001). After adjustment for gender and age, hypertension and dyslipidemia, a greater prevalence of osteoarticular, respiratory and psychiatric diseases was noted in both AD and Pso patients, whereas increased prevalence of obesity was seen only in Pso patients. The prevalence of components of metabolic syndrome was higher among Pso than AD patients., Conclusion: Further studies are required to consolidate these findings, to better characterize the entire spectrum of AD and Pso comorbidities, and to better identify determinants and risk factors, along with targeted therapies., (Copyright © 2020. Published by Elsevier Masson SAS.)

Objective: Although several scores exist to assess psoriasis severity, most have marked limitations that rule out their use in routine clinical practice. A new score, the Simplified Psoriasis Index (SPI), has recently been developed and validated in adults in Britain for such use. It has separate components for current severity (SPI-s), psychosocial impact (SPI-p) and past history and interventions (SPI-p), and it is suitable for either professional assessment or patient self-assessment. The aim of this work was to produce a validated translation of SPI into French (as spoken in France)., Methods: The index was translated and validated using a strict methodology comprising respectively five and eight phases for the professional (proSPI) and self-administered instruments (saSPI). Translation of the saSPI instrument also involved a cognitive debriefing with five psoriasis patients., Results: Linguistic discrepancies and subtle differences of meaning arising during the process were closely examined. The developer of the instrument ensured conceptual accuracy. A panel of health experts guaranteed that medical terms were correctly translated. Five patients with plaque psoriasis (two female and three male of median age 45 years [range: 31-78]) tested the SPI-p version during cognitive interviews and found the questionnaire clear and easy to understand., Conclusion: Validated French translations of both SPI instruments are now available for use in routine clinical practice. Further investigations are currently underway to validate the psychometric properties of the instrument., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Background: Permethrin is a synthetic pyrethroid used as a chemical insecticide that obtained an MA in the management of human scabies in 2014. We report a case of severe immediate hypersensitivity (IH) reaction with generalized contact urticaria secondary to the cutaneous application of 5% permethrin cream (Topiscab®)., Observation: A 44-year-old woman with no personal history of atopy was treated with oral ivermectin, Topiscab® and levocetirizine for suspected scabies. Eight hours after taking a levocetirizine tablet and five hours after the application of a tube of Topiscab® together with oral ivermectin, she presented generalized urticaria, nausea and diarrhoea, followed by loss of consciousness. Skin prick-tests for ivermectin and levocetirizine were negative. We noticed non-significant erythema with permethrin. The open application test with Topiscab® was strongly positive at 20min with the appearance of an urticaria plaque in the area of application. The open test with sorbic acid was positive at 2h. Accidental exposure to permethrin spray caused dyspnoea and recurrence of urticaria., Discussion: Mild and transient symptoms are regularly described after cutaneous application (burning, paraesthesia or increased itching). Delayed hypersensitivity reactions such as contact dermatitis have been reported in the literature. Exceptional cases of severe IH reactions have been described following occupational exposure to airborne pyrethroid insecticides. No cases of severe IH reaction secondary to application of topical permethrin have been reported., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Background: Anti-PD-1 and anti-CTLA-4 monoclonal antibodies are used in melanoma, while anti-PD-1 are also used in Hodgkin's lymphoma. Primary central nervous system lymphoma is a rare form of non-Hodgkin's lymphoma with few effective treatments. However, several recent studies have reported multiple cases of non-Hodgkin's lymphoma and primary central nervous system lymphoma treated by anti-PD-1 antibodies with favourable responses., Patients and Methods: This study focuses on the case of a 59-year-old man with metastatic melanoma treated by immunotherapy (anti-CTLA-4 followed by anti-PD-1). He underwent 28 courses of therapy with pembrolizumab. Treatment was stopped after clinical and radiological remission. The patient presented left hemiparesis and a primary central nervous system lymphoma was diagnosed two months after discontinuation of immunotherapy. He started urgent high-dose methotrexate chemotherapy but without significant results. Despite second-line chemotherapy with R-ICE (rituximab-ifosfamide, carboplatin and etoposide), the patient died., Discussion: Several hypotheses may be advanced regarding a possible relationship between immunotherapy and the occurrence of this primary central nervous system lymphoma. The lymphoma may have been pre-existing and controlled by immunotherapy, but progressing rapidly after treatment, or it may have been induced by the immunotherapy. However, immunotherapy may have played no role; the relationship between melanoma and lymphoma is well known., Conclusion: While immunotherapy cannot be unequivocally incriminated in primary central nervous system lymphoma, this case raises many questions about the imputability of immunotherapy in the occurrence of secondary cancers, including lymphomas., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Introduction: Atopic keratoconjunctivitis is associated with eyelid eczema. It may require the use of local corticosteroids which if prolonged can be a source of ocular complications. Tacrolimus is an immunosuppressant used in cutaneous application in atopic dermatitis. The aim of this study was to measure the efficacy and tolerance of tacrolimus 0.1% ointment in palpebral application in atopic keratoconjunctivitis., Patients and Methods: This retrospective, single-center study was conducted between June 2014 and February 2017. Patients with atopic keratoconjunctivitis not controlled by first-line medical treatments were included. The primary endpoint was the evolution of functional signs as assessed by the NEI-VFQ25 and OSDI quality of life scores. Secondary endpoints were visual acuity and local corticosteroid use., Results: Among the 18 patients included, the mean age was 37.9±16.8 years. The first follow-up visit was on average 68.3±55.3 days after initiation of treatment. The NEI-VFQ25 score was significantly improved for seven of its sub-scores (P<0.05) and the mean OSDI decreased significantly from 52.3±26.2 to 22.0±27.0 (P<0.001) showing a decrease in eye discomfort. A significant reduction was observed in the number of patients using local corticosteroids. There was no significant change in visual acuity., Conclusion: Tacrolimus ointment 0.1% in palpebral application appears to be an effective treatment for the management of atopic keratoconjunctivitis., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Introduction: Atopic keratoconjunctivitis is frequently associated with atopic eyelid dermatitis. It may require topical steroids, the prolonged use of which may cause ocular complications. Tacrolimus is an immunosuppressant used topically on the skin in atopic dermatitis. The purpose of this study is to evaluate the efficacy and tolerability of tacrolimus 0.1% ointment applied to the eyelids in atopic keratoconjunctivitis., Patients and Methods: This is a single center, retrospective study carried out between June 2014 and February 2017. Patients presenting with atopic keratoconjunctivitis uncontrolled by first-line medical treatment were included. The main outcome was change in functional symptoms as evaluated by the NEI-VFQ25 and OSDI quality of life scores. Secondary criteria were visual acuity and topical steroids use., Results: Among the 18 patients included, the mean age was 37.9±16.8years. The first follow-up visit occurred on average 68.3±55.3 days after initiation of treatment. The NEI-VFQ25 score improved significantly for seven of the sub-scores (P<0.05), and the mean OSDI decreased significantly from 52.3±26.2 to 22.0±27.0 (P<0.001), demonstrating a decrease in ocular symptoms. A significant reduction in the number of patients requiring topical steroid treatment was observed. There was no significant change in visual acuity., Conclusion: Tacrolimus 0.1% ointment applied to the eyelids appears to be an effective treatment in the management of atopic keratoconjunctivitis., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Background: Food urticaria is common and generally benign, and it may be of viral or idiopathic aetiology. A food origin of the allergy is frequently sought but rarely found. Mammalian meat anaphylaxis, or alpha-galactose (α-gal) anaphylaxis, is a rare and recently discovered entity., Patients and Methods: Herein, we report a case of alpha-galactose (α-gal) anaphylaxis in a 60-year-old woman presenting four episodes of acute urticaria with signs of anaphylaxis occurring a few hours after meals containing mammalian meat (beef meat, pork meat and offal). The diagnosis was confirmed by a positive gelatine prick-test and the presence of α-gal IgE., Discussion: In the event of acute urticaria associated with systemic symptoms, in particular gastrointestinal signs, allergy to α-galactose should be considered., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

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., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

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., (Copyright © 2016 Elsevier Masson SAS. All rights reserved.)

Objective: To assess the rate of complications of radical lymph node dissection following sentinel lymph node biopsy in patients with malignant melanoma., Patients and Methods: A single-centre retrospective study was performed between 2001 and 2009. All melanoma patients who first had a sentinel node biopsy followed by a radical lymph node dissection were included., Results: Seventy-four patients had radical lymph node dissection after confirmation of metastasis in a sentinel node. The sites of the lymphadenectomy were axillary in 51% of patients, groin in 43%, neck in 5% and popliteal in 1%. A nodal metastasis was observed in 12 of 74 lymph node dissections. Median follow-up time was 41.5 months (3-135 months). At least one complication was seen in 47 patients (64%). Early complications were: postoperative infection (n=9; 12%), lymphocele (n=22; 30%), chronic seroma (n=9; 12%), postoperative haematoma (n=6; 8%), and wound separation (n=7; 9%). Late complications were: lymphoedema (n=30; 41%), joint complications (n=10; 14%), fibrous scar (n=11; 15%), chronic pain (n=6; 8%) and hyposensitivity (n=6; 8%). Two patients were rehospitalised for a complication. The complication rate was 53% for axillary location and 81% for inguinal location (P=0.02)., Conclusion: Radical lymph node dissection performed after positive sentinel node biopsy in melanoma patients is associated with significant morbidity., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

Background: Surgery is the standard treatment for vulvar Paget's disease, despite its mutilating consequences and the high associated recurrence rate. Alternative treatments such as CO₂ laser, application of imiquimod and topical photodynamic therapy (PDT) have recently been proposed. We report on two patients with vulvar Paget's disease treated with combined cryosurgery and PDT., Patients and Methods: Case 1: a 40-year-old pregnant woman presented large vulvar lesions suggestive of Paget's disease. After imiquimod treatment failed, three sessions of cryosurgery were performed on all lesions at 5-month intervals, followed by PDT sessions for the residual erythema. Because of recurrent focal lesions, further sessions of localized cryosurgery were performed for 18 months. Case 2: a 61-year-old patient with extensive vulvar Paget's disease relapsed after imiquimod treatment and was treated by cryosurgery and multiple PDT sessions. She was in complete remission 12 months after this treatment., Discussion: Combined cryosurgery and PDT may offer a therapeutic alternative to conventional surgery for the treatment of large or recurrent multifocal vulvar Paget's disease, or in elderly people. Indeed, although palliative, these two non-invasive techniques can be used to treat many lesions in a single session. Recurrences can be treated again using either technique, or both, without any aesthetic or functional consequences., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

Background: The aim of this study was to assess changes in the incidence and mortality rates of malignant melanoma (MM) over a 20-year period in a region without a cancer registry., Methods: All cases of MM studied were collected retrospectively from the databases of six private and three hospital-based histopathology laboratories in the Seine-Maritime region covering three 24-month periods each 10years apart: 1988-1989, 1998-1999 and 2008-2009, The incidence and mortality rates were estimated based on data provided by French National Institute for Statistics and Economic Studies (Insee) and French National Institute of Health and Medical Research (Inserm)., Results: Over the 20-year period, the incidence of MM increased from 8.6 to 21.2/100,000inhabitants per year (+147%, P<0.0001) while the mortality rate rose from 1.3 to 2.8/100,000inhabitants per year (+115%, P=0.0003). The incidence of invasive MM increased by +110%, while the incidence of MM in situ increased by +456%. The incidence and overall mortality rate of invasive MM increased particularly during the first 10-year period: +62% (P<0.0001) and +77% (P=0.01) respectively, and to a much lesser extent during the last 10-year period: +30% (P=0.0007) and +22% (P=0.22) respectively. This slowdown in the incidence of invasive MM and in overall mortality rates was even more pronounced in women over the last 10years (+17 and +9%), whereas these rates continued to increase in men (+49% and +35%, respectively). In contrast, the incidence of MM in situ increased above all during this same period (+257%)., Conclusion: This study shows that while the incidence and mortality rate of invasive MM has increased little over the last 10years in the Seine-Maritime region, the incidence of MM in situ continues to rise sharply., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

Background: Types of subepidermal autoimmune bullous dermatosis (AIBD) are classified by anatomoclinical picture and target antigen. A new entity has recently been identified: anti-p200 pemphigoid., Patients and Methods: An 82-year-old man consulted for a profuse pruritic bullous eruption refractory to the standard treatments for bullous pemphigoid (BP). Direct immunofluorescence examination of a skin biopsy revealed linear deposits of IgG and of C3 at the dermal-epidermal junction, but Elisa screening for circulating anti-BP180 and anti-BP230 antibodies was negative. Indirect immunofluorescence (IIF) testing of cleaved skin revealed a deposit of IgG4 antibodies on the dermal side. Immunoblotting was negative for a dermal extract but showed an antibody directed against a 200-kD epidermal antigen. A diagnosis of anti-p200 pemphigoid was eventually made and the patient was successfully treated with dapsone., Discussion: The diagnosis of anti-p200 pemphigoid was made in this case in spite of discrepancy between the IIF and immunoblotting results, and despite the fact that the target antigen in this disease is considered as being restricted to dermal sites. Anti-p200 pemphigoid usually begins in the second part of life and differs from standard bullous pemphigoid in terms of more frequent mucous membrane and cephalic involvement, as well as a greater degree of miliary scarring. This disease appears more prominent in males and is associated with psoriasis in around one third of cases. Autoantibodies recognize laminin gamma-1, an extra-desmosomal protein that contributes to dermal-epidermal adhesion., Conclusion: This recently described disease as probably under-diagnosed in France. It should be considered in atypical presentations of bullous disease. Diagnosis is confirmed by immunoblotting detection of autoantibodies directed against a 200-kD antigen normally present in the extract. Dapsone appears to be the most effective treatment., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

Background: Pemphigus is a rare autoimmune bullous disorder. Numerous treatment regimens have been proposed in the literature., Objective: To assess the efficacy and tolerance of treatment regimens proposed in pemphigus vulgaris (PV) and pemphigus foliaceus (PF), from a systematic review of the literature., Methods: Randomized control trials have been identified using the PubMed and Embase databases up to April 2009. Uncontrolled prospective and retrospective studies have also been analyzed., Results: Eleven randomized control trials having included a total number of 421 patients (377 PV, 44 PF) have been analyzed. Most studies had a limited statistical power due to the rather low number of cases included. Results from ten different treatment regimens have been analyzed: different dosages of prednisone and prednisolone, pulse intravenous dexamethasone, azathioprine, cyclophosphamide, cyclosporine, dapsone, mycophenolate mofetil, plasmapheresis, topical applications of epidermal growth factor (EGF), and intravenous immune globulins (IVIG). Inclusion criteria were: (i) consecutive patients in nine studies, (ii) patients who did not respond to low doses of corticosteroids in one study, and (iii) patients with relapsing type of pemphigus in one study. None of these studies allowed identifying the best effective and well tolerated regimen. Mycophenolate mofetil was more effective than azathioprine for disease control (from one study; n=40; OR=0.72; 95% CI=0.52-0.99). However, no difference in the rate of clinical remission was evidenced between these drugs. Azathioprine and cyclophosphamide seem to have a corticosteroid sparing effect., Conclusion: Data from the literature did not allow identifying the best therapeutic regimen, mainly because of the lack of statistical power of most studies. The usefulness of immunosuppressant added to systemic corticosteroids as the first line of treatment is not clearly established., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

Background: Treatment of severe and chronic forms of alopecia areata (AA) totalis remains difficult. It has been suggested that methotrexate (MTX) is an efficacious treatment for AA totalis. The aim of our study was to assess the long-term safety and efficacy of MTX in patients with chronic severe forms of AA totalis., Patients and Methods: Thirty-three patients (24 women and nine men) with AA totalis or universalis were included. Mean disease duration was 7.7 years and these patients had not responded to prior standard treatment. The initial weekly dosage of MTX was 15 mg (n=2), 20mg (n=16) or 25mg (n=15). Nineteen patients (58%) were treated with MTX and low doses of corticosteroids (prednisone 10-20mg/d), while 14 patients (42%) received MTX alone. The primary end-point was complete hair regrowth under treatment., Results: Twenty-one patients had total hair regrowth and 13 patients were considered as treatment failures, with 10 of these having partial regrowth and three having no regrowth. Complete regrowth was seen in 12 of 19 patients (63%) on combined treatment and in eight of 14 patients (57%) on MTX alone. The onset of hair regrowth was noted after a median delay of 3 months. At the end of the study, after a median of 30 months' follow-up, the median cumulative dose of MTX was 1.8 g (400mg-5.5 g). Relapse was observed on corticosteroid dose reduction or withdrawal in 16 of the 20 patients in whom total hair regrowth initially occurred. Regrowth after treatment of relapse was seen in 14 of these 16 patients (87%). Seven patients experienced adverse events consisting of transient elevated transaminases (n=4), persistent nausea (n=2) and lymphocytopenia (n=1)., Conclusion: MTX alone or in combination with low doses of oral corticosteroids resulted in complete hair regrowth in about half of these patients presenting AA totalis or universalis. Lasting improvement required continuous treatment in most cases., (Copyright 2010 Elsevier Masson SAS. All rights reserved.)

Background: Birt-Hogg-Dubé syndrome (BHDS) is an autosomal-dominantly inherited genodermatosis that predisposes to the development of benign hair follicle tumours, lung cysts, kidney tumours, and possibly colonic cancers, due to mutations in the FLCN gene. We report cases involving a new mutation in three unrelated families., Materials and Methods: Blood samples of three probands were submitted for a molecular diagnosis of BHDS. Following DNA extraction, FLCN gene sequencing was performed. The identified mutations were confirmed on a second sample. A cancer genetics consultation was organized and specific tests (dermatological examination, CT scan of chest and abdomen and colonoscopy) were proposed for each BHDS patient., Results: FLCN gene-sequencing analysis revealed an identical complex harmful mutation in all three families. The first proband showed fibrofolliculomas (FF), a history of pneumothorax and colonic adenoma. The mutation was found in a brother and two sisters, who were asymptomatic, and in a niece with FF. The second proband showed FF. The mutation was found in her mother, who had FF. The third proband presented diffuse emphysema and very rare FF., Discussion: This case report shows extremely wide intra- and interfamilial phenotype variation within individuals having a similar FLCN gene mutation. In large cohorts of BHDS patients, no genotype-phenotype correlation has been shown. This case emphasises the vital importance of presymptomatic diagnosis for each member of a BHDS family by means of a cancer genetics consultation, followed by a CT scan of the chest and abdomen, colonoscopy and annual kidney imaging., (Copyright 2010. Published by Elsevier Masson SAS.)

Background: Bullous pemphigoid (BP) is the most common form of autoimmune bullous dermatosis. The first signs of the disease comprise non-specific lesions. We conducted a retrospective study of two large series of cases of BP to identify parameters related to patients or to the disease affecting time to diagnosis (TD)., Methods: We used files from the "BP1" series retrospectively (comparison of topical and oral corticosteroids) and from the "BP2" series (comparison of two regimens of topical corticosteroids) in order to determine mean and median TD. In each series, patients were divided into two equivalent groups, i.e. patients with short TD (STD), i.e. less or equal to median, and patients with long TD (LTD), i.e. greater than median. Patient-related parameters (age, Karnofsky score, associated neurological diseases) and disease-related parameters (number of blisters and eosinophil count at diagnosis) were compared between these groups. We also investigated for any influence on TD of dermatological demography., Results: Mean TD in the BP2 series was significantly shorter than in the BP1 series (61 versus 91 days, p=0.04) but the median values were similar (31 versus 36 days). There was no difference in patient age, associated neurological diseases or numbers of bullae and eosinophils between the two groups. In contrast, in the BP2 series alone, Karnofsky score was significantly lower in patients with STD (60.25%) than in patients with LTD (66%), p=0.02. Dermatological demographics had no effect on TD., Conclusion: Karnofsky score is the only parameter that appears to be associated with TD, with patients with worse scores having a shorter TD, probably due to better medical follow-up. Improved knowledge among general practitioners concerning the initial signs of the disease should shorten TD and accelerate the institution of appropriate therapy.

Background: Imatinib (Glivec) is a tyrosine kinase inhibitor used to treat certain cases of leukaemia. We report a case of a drug-induced reaction with eosinophilia and systemic symptoms (DRESS) caused by imatinib., Case-Report: A 77-year-old woman with a chronic myeloid leukaemia was treated with imatinib and allopurinol. Nineteen days after the start of treatment, the patient presented fever with a generalized polymorphous rash associated with oral erosions, facial oedema, diffuse lymphadenopathy and blood hypereosinophilia. Histological analysis of skin biopsy specimens suggested a drug-induced reaction. The outcome was favourable two weeks after discontinuation of treatment. Three months later, imatinib was reintroduced because of progression of the patient's chronic myeloid leukaemia, and recurrence of the skin rash and fever was observed within 12 hours., Discussion: Allopurinol was stopped definitively because of its more frequent imputability. Imatinib was reintroduced after considering the benefit-risk ratio and in full knowledge of the existence of cutaneous reactions to imatinib, despite there being only one recent report of DRESS following treatment with imatinib. According to the causality criteria of Bégaud et al. regarding imatinib, inherent causality of the drug in our patient was initially possible (I2) and appeared likely (I3) after the rechallenge test. This case clearly illustrates that imatinib is a potential cause of DRESS.

Background: Lymphomatoid papulosis is a form of CD30+ cutaneous lymphoproliferation characterized by a benign chronic papulonodular eruption that regresses spontaneously. The clinical features contrast with the malignant histological aspect of lesions. Mucosal lesions are rare, with less than 10 published cases. We report four new cases and we highlight characteristic features of lesions at this particular site., Patients and Methods: We report four cases of mucous lymphomatoid papulosis in three women aged 37, 38, and 71 years and one 66-year-old man. These cases were collated from three different hospitals: Orléans, Rouen and Caen. Mucosal lesions occurred after cutaneous eruption in two cases but remained isolated or preceded cutaneous lesions in the other two cases. The main site was the mouth in all four cases but one case also involved genital lesions. Two cases involved type A pathological features and two had type C features. Association with lymphoma was excluded on clinical, laboratory and radiological examination. One patient was treated with methotrexate (>7.5mg/week) and did not relapse. Of the three other untreated patients only one did not relapse (short 14-month follow-up)., Discussion: Recurrent oral ulcerations may be mucosal manifestations of lymphomatoid papulosis. This site does not appear to have any bearing on prognosis.