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Summary Background Generalized bullous fixed drug eruption (GBFDE) is a rare cutaneous adverse reaction to drugs, and may resemble Stevens–Johnson syndrome (SJS) or toxic epidermal necrolysis (TEN), but is usually considered less severe. Objectives To compare the severity and mortality rate in cases of GBFDE and control cases of SJS or TEN of similar extent of skin detachment. Methods This was a case–control analysis of 58 patients with GBFDE matched by age and extent of skin detachment to 170 control patients with a validated diagnosis of SJS or SJS/TEN overlap. Data for cases and controls were extracted from the EuroSCAR and RegiSCAR databases resulting from two population-based studies of severe cutaneous adverse reactions conducted in Europe. Preselected outcome criteria were death (primary), and fever, duration of hospitalization and transfer to an intensive care or burn unit (secondary). Results GBFDE affected mainly older patients (median age 78 years, interquartile range 68–84 years); 13 of 58 cases died (22%). The mortality rate was slightly but not significantly lower for patients with GBFDE than controls [28%, multivariate odds ratio 0·6 (95% confidence interval 0·30–1·4)]. Patients with GBFDE and controls did not differ in other preselected criteria for severity. Conclusions Although our study featured limited statistical power, we were not able to confirm that GBFDE had better prognosis than SJS or SJS/TEN of similar disease extent in older patients. Severe cases of GBFDE deserve the attention and active management given to patients with SJS or TEN.

Background: The value of 230-kDa bullous pemphigoid antibody (BP230) enzyme-linked immunosorbent assay (ELISA) for the diagnosis of bullous pemphigoid (BP) was investigated, but in the immunological follow-up of the disease remains unknown. Objective: Evaluation of BP230 ELISA for diagnosis, follow-up and prediction of relapse in BP. Methods: Monocenter retrospective and prospective study. Patients with typical BP. Detection of autoantibodies by indirect immunofluorescence (IIF), BP180 and BP230 ELISA tests at diagnosis, during the treatment (disease control or failure) and at treatment stop (relapse or not 3 months after). Results: 74 patients were included. At diagnosis, BP230 ELISA sensitivity was lower than IIF and BP180 ELISA. Combining both ELISA added a weak gain of sensitivity. Both tests paralleled the clinical evolution, especially in case of disease control. At the end of the treatment, BP230 ELISA was not different in patients with or without relapse. Conclusion: In routine practice, BP230 ELISA does not seem to be a useful additional test in typical BP.

Introduction L’acronyme DRESS rend compte des caracteristiques d’un type original de reaction medicamenteuse. Les DRESS s’accompagnent (jusqu’a 60 % des cas) de reactivation d’un ou plusieurs virus « Herpes » (CMV, EBV, HHV6, HHV7). Pour certains le « DRESS n’est qu’une maladie virale » induite par effet direct des medicaments. Methodes Revue de la litterature (Pubmed) depuis 1955 sur immunite cellulaire et reactivation virale. Resultats Lymphocytes, monocytes, macrophages sont reservoirs d’herpes virus latents. Pour obtenir des particules HHV6 les virologues stimulent par PHA ou PMA des cellules mononuclees d’adultes sains. La reactivation virale par stimulation lymphocytaire a fonde un test (« virus plaque assay ») pour compter les lymphocytes specifiques d’antigene. Une couche de cellules sensibles a la lyse virale est couverte de lymphocytes prealablement infectes par un virus cytolytique et stimules par l’antigene a tester. Le nombre de plaques de lyse quantifie les lymphocytes specifiques. Tests epi-cutanes, et tests in vitro sont plus souvent et plus fortement positifs dans les DRESS, expliquant la reactivation virale plus frequente. Discussion Ces faits oublies montrent que le DRESS est primitivement une hypersensibilite medicamenteuse dont decoule parfois une reactivation virale. Cette reactivation est-elle pathogene ? Chez l’adulte sain les reactivations virales sont reputees non rares et asymptomatiques. Chez les immunodeprimes des manifestations graves (encephalites, colites ulcerees…) peuvent en resulter. Ces complications sont exceptionnelles dans les DRESS. Deux series retrospectives de « tailles » limitees comparant DRESS avec et sans reactivation ont suggere que fievre, evolution prolongee, adenopathies, lymphocytes atypiques et hepatite etaient plus frequents en cas de reactivation. Une etude prospective de large taille pourra seule evaluer l’impact reel des reactivations virales du DRESS. Conclusion Le DRESS est primitivement une manifestation severe d’hypersensibilite medicamenteuse. La reactivation virale n’est qu’un marqueur de cette l’intensite. Sa possible contribution aux symptomes et a la severite des DRESS reste a evaluer.

Resume Introduction Le syndrome d’hypersensibilite medicamenteuse ou drug reaction with eosinophilia and systemic symptoms (DRESS) est une toxidermie severe dont la physiopathologie implique la reactivation de virus Herpes. Nous rapportons deux cas de thyroidite developpes dans les suites d’un DRESS, sans antecedent connu de maladie auto-immune, et associes a une reactivation virale HHV6. Observations Cas 1 : Une femme de 39 ans etait hospitalisee pour un DRESS avec une reactivation HHV6 prolongee et une atteinte cutanee, ganglionnaire, hepatique et renale necessitant une corticotherapie generale prolongee. Elle developpait a l’arret de ce traitement une maladie de Basedow, qui etait stabilisee par la suite sous carbimazole et beta-bloquant. Cas 2 Une femme de 31 ans etait adressee pour un DRESS, avec reactivation virale HHV6 retardee, complique d’atteinte hepatique. A six semaines, une rechute cutanee et une thyroidite de De Quervain etaient observees. Un traitement par levothyroxine sodique etait debute ainsi que des dermocorticoides de classe tres forte, permettant une evolution favorable. Discussion L’implication de reactivations virales, notamment de HHV6, est actuellement discutee dans les formes prolongees, les rechutes et le developpement de maladies auto-immunes. Ces observations soulignent le risque potentiel de maladies auto-immunes et la necessite d’une surveillance clinique, biologique reguliere et prolongee des patients ayant fait un DRESS.

Background: The efficacy of topical steroids in bullous pemphigoid (BP) was assessed by prospective therapeutic trials. Systemic corticosteroids and immunosuppressant agents are indicated in situations of failure or relapses. Objectives: To report our experience in the management of BP outside therapeutic trials focusing on the outcome of the patients at 6 and 12 months, compliance and follow-up difficulties. Methods: Monocenter retrospective study with collection of clinical, therapeutic and follow-up data after 6 and 12 months. Results: Ninety-six patients, mean age 84 years, 65 females, 54% had neurological impairment. The initial dose of clobetasol propionate was 30 g/day, followed by a progressive decrease. In the first 6 months, 14% were lost to follow-up and 17.7% died; 62% were controlled with topical steroids alone, and 25% had adjunctive systemic treatment. Difficulties of compliance were mentioned in 34.4%, without significant difference between controlled and noncontrolled patients. After 12 months, 23% were lost to follow-up and 27.1% died. The mean duration of the treatment was 11.7 ± 8.4 months. After the stop, 18.9% of patients relapsed within 3 months. Conclusion: We emphasize the frequent recourse to systemic treatments in the first few months, the difficulties of compliance and of follow-up in our day-to-day experience.

Resume Introduction Les abces du psoas sont une entite clinique rare. Ils sont le plus souvent secondaires a un foyer infectieux intra-abdominal ou retroperitoneal. Nous rapportons deux cas d’abces du psoas a streptocoque beta-hemolytique du groupe A secondaires a une porte d’entree cutanee. Observations Le premier patient, âge de 50 ans, etait febrile et presentait des lesions cutanees ulceronecrotiques evocatrices d’ecthyma evoluant depuis trois mois, associees depuis peu a une masse douloureuse de la fosse iliaque gauche entrainant des difficultes a la marche. La seconde patiente, une femme de 35 ans aux antecedents de toxicomanie intraveineuse, etait transferee en reanimation dans un contexte de sepsis severe a streptocoque beta-hemolytique du groupe A de localisation initiale cutanee (placard inflammatoire du membre inferieur gauche). Elle restait subfebrile dix jours apres le debut d’une antibiotherapie par amoxicilline, sans point d’appel infectieux. Un examen tomodensitometrique abdominopelvien confirmait le diagnostic d’abces du psoas gauche chez les deux patients. Pour le premier, le meme streptocoque beta-hemolytique du groupe A etait isole a la fois sur le liquide de drainage et sur les lesions cutanees. L’evolution etait favorable dans les deux cas sous antibiotherapie intraveineuse prolongee (amoxicilline) et drainage percutane (premier patient seulement). Discussion L’abces du psoas peut etre secondaire, par contiguite avec un foyer infectieux locoregional. La porte d’entree initiale est alors le plus souvent digestive, articulaire, gynecologique ou urologique et les germes en cause sont varies. L’abces du psoas peut aussi etre primitif ; le microorganisme le plus frequemment retrouve est alors le staphylocoque dore. Dans nos deux observations, il s’agit d’abces du psoas secondaires a une porte d’entree cutanee. La triade evocatrice de fievre prolongee, douleur et psoitis est souvent tardive et il faut savoir evoquer le diagnostic devant un etat infectieux prolonge ou une douleur abdominale febrile inexpliquee. Le diagnostic de certitude repose sur la tomodensitometrie abdominale. Le traitement fait generalement appel a une antibiotherapie intraveineuse a large spectre associee a un drainage percutane ou chirurgical. Le taux de mortalite de ces patients reste eleve et la survie est conditionnee par la rapidite de la prise en charge.

Resume Introduction Le diagnostic d’endocardite infectieuse (EI) est souvent suspecte cliniquement, mais reste particulierement difficile a confirmer. Les manifestations dermatologiques de l’endocardite sont variees et doivent etre recherchees de facon systematique dans un contexte de suspicion d’EI. Le but de cette etude etait d’etudier les modalites de revelation de l’EI dans un service de dermatologie. Malades et methodes Nous avons inclus retrospectivement l’ensemble des patients hospitalises de facon consecutive dans le service de dermatologie de l’hopital Henri-Mondor entre mai 2006 et mai 2007, pour lesquels un diagnostic d’EI avait ete pose selon les criteres de Duke. Resultats Sept malades ont ete inclus, d’un âge moyen de 61 ans. Les motifs d’hospitalisation en dermatologie etaient : ulceres chroniques des membres inferieurs ( n = 2), syndrome de Sezary ( n = 1), epidermolyse bulleuse acquise ( n = 1), dermatite atopique ( n = 1), purpura ( n = 1). Les manifestations dermatologiques de l’EI comprenaient : purpura ( n = 5), necroses cutanees distales ( n = 2), hemorragies sous-ungueales ( n = 1). Les hemocultures etaient positives dans trois cas ( Staphylococcus aureus sensible [ n = 2] ou resistant [ n = 1] a la meticilline). La serologie Coxiella burnetti etait positive dans un cas. La porte d’entree etait cutanee dans six cas, sur une dermohypodermite chronique ou une dermatose chronique a peau colonisee ( n = 3), une perfusion peripherique ( n = 2), un catheter d’hemodialyse ( n = 1). L’echographie transthoracique etait negative six fois sur sept, l’echographie transœsophagienne permettant de confirmer le diagnostic dans cinq cas sur six avec un delai diagnostique moyen de 21 jours. La localisation de l’EI etait la valve mitrale ( n = 3), la valve aortique ( n = 2), les valves mitrale et aortique ( n = 1), la sonde de pacemaker ( n = 1). Cinq patients sont decedes dans un delai moyen de 78 jours. Conclusion Le diagnostic d’EI reste particulierement difficile. Il doit etre discute devant des signes cutanes comme un purpura ou des necroses distales…, mais egalement devant des dermatoses chroniques partiellement controlees, voire refractaires aux traitements, qui constituent des portes d’entree a ne pas sous-estimer. Cette etude souligne la necessite d’une vigilance accrue des cliniciens lors de la prise en charge de patients colonises ou subissant des gestes invasifs sur peau lesee.

Resume Introduction La tularemie est une anthropozoonose transmise par des petits mammiferes (lievre) et des insectes (tiques, taons). L’agent responsable est Francisella tularensis , bacille Gram negatif intracellulaire facultatif. Nous rapportons un cas de tularemie dans sa forme ulceroganglionnaire survenue au cours du traitement d’une polyarthrite rhumatoide par l’adalimumab, un anticorps monoclonal anti-TNF alpha. Observation Un homme de 58 ans consultait pour un placard inflammatoire de la jambe gauche centre par une zone necrotique, associe a une fievre et une adenopathie inguinale homolaterale. Il avait pour antecedents une primo-infection tuberculeuse et une polyarthrite rhumatoide traitee depuis un an par methotrexate et adalimumab. L’interrogatoire precisait la notion d’une morsure de tique quelques semaines auparavant. Une antibiotherapie par doxycycline etait prescrite, mais l’adenopathie evoluait progressivement vers l’aggravation et la fistulisation. L’exerese du ganglion etait finalement pratiquee. L’examen histologique revelait une lymphadenite granulomateuse epithelioide et gigantocellulaire avec necrose ; la recherche de mycobacteries en culture etait negative. La serologie de la tularemie etait positive a un taux significatif et le diagnostic de tularemie ulceroganglionnaire apres morsure de tique etait retenu. Ce diagnostic etait confirme secondairement par detection de l’ADN de F. tularensis sur la biopsie ganglionnaire. La doxycycline etait poursuivie pendant six semaines. L’infection n’a pas recidive apres un an de recul. Discussion Une adenopathie febrile survenant sous anti-TNF alpha et prenant un aspect histologique de granulome tuberculoide avec necrose evoque en premier lieu une reactivation ganglionnaire de tuberculose. Cependant, la notion de morsure de tique et l’impossibilite d’isoler des mycobacteries a partir des multiples prelevements ont impose la recherche d’une maladie d’inoculation et conduit finalement au diagnostic de tularemie. Le role favorisant des therapeutiques immunosuppressives dans la survenue de cette forme grave de tularemie doit etre evoque.

Resume Introduction La pemphigoide bulleuse (PB) peut debuter par des signes non specifiques trompeurs. Nous avons realise une etude retrospective sur deux grandes series de PB pour rechercher des parametres associes au delai diagnostique (DD). Materiel et methodes Nous avons utilise retrospectivement les fichiers des etudes « PB1 » (comparaison corticotherapies locale/generale) et « PB2 » (comparaison corticotherapies locales allegee/forte) pour calculer le DD moyen et median de la maladie. Chaque groupe de patients a ete divise en deux sous-groupes egaux, celui a DD inferieur ou egal a la mediane (DDC) et celui a DD superieur a la mediane (DDL). Nous avons compare entre ces groupes des parametres lies au patient (âge, indice de Karnofsky, antecedents neurologiques) et a la maladie (nombre de bulles et de polynucleaires eosinophiles). Nous avons recherche un effet centre (comparaison des DD selon le nombre de patients inclus) et un role de la demographie dermatologique regionale. Resultats Le DD moyen des patients de « PB2 » etait significativement inferieur a celui de « PB1 » (61 versus 91 jours, p = 0,04) mais les medianes (31 versus 36 jours) etaient equivalentes. L’âge, les antecedents neurologiques, le nombre de bulles et de polynucleaires eosinophiles n’etaient pas significativement differents entre les groupes. Seul l’indice de Karnofsky etait significativement plus bas dans le groupe PB2-DDC (60,25 %) que dans le groupe PB2-DDL (66 %), p = 0,02. Aucun effet centre, ni impact de la demographie dermatologique n’etait mis en evidence. Conclusion Seul l’indice de Karnofsky semble etre associe au DD, refletant une plus grande medicalisation des patients les moins valides. Une meilleure connaissance de la PB par les medecins traitants pourrait permettre de raccourcir le DD et accelerer l’institution d’un traitement adapte.

Purpose To investigate whether propensity score (ps) methods could reasonably be applied to estimate the treatment effect on mortality, based on a comparatively small sample of patients with severe cutaneous adverse reactions (SCAR) and who come from different countries where physicians prefer different treatment schemes. Methods Ps methods were applied to cope with confounding due to non-randomized treatment assignment for the analysis of the treatment data obtained in the case-control study EuroSCAR. For the study's purpose, the analysis focused on the comparison of the treatments: corticosteroids (STER) and supportive care only (SUPP). Results 206 French and German patients were treated either with SUPP or STER. Imbalances between treatment groups as well as between the countries were recognized. Concerning the balance between the treatment groups no ps model for the full cohort was satisfying. In addition, the inclusion of a variable for patient's country led to a separation of the patients by country. Thus, we developed ps models for each country separately and estimated the treatment effects (France: odds ratio (OR) 0.52, 95% confidence interval (CI) 0.09–3.10, Germany: OR 0.23, CI 0.06–0.92, Overall: OR 0.33 CI 0.11–1.04). Conclusions The application of the ps methods was successful and provided valuable information. We could confirm the findings of the original analysis which was based on standard logistic regression, especially concerning the necessity of a country-specific analysis. The observed country differences in the estimated treatment effects were less pronounced and thus seemed to be more reasonable than those of the past analysis. Copyright © 2009 John Wiley & Sons, Ltd.

Background Acute generalized exanthematous pustulosis (AGEP) is a disease characterized by the rapid occurrence of many sterile, nonfollicular pustules usually arising on an oedematous erythema often accompanied by leucocytosis and fever. It is usually attributed to drugs. Objectives To evaluate the risk for different drugs of causing AGEP. Patients and methods A multinational case-control study (EuroSCAR) conducted to evaluate the risk for different drugs of causing severe cutaneous adverse reactions; the study included 97 validated community cases of AGEP and 1009 controls. Results Strongly associated drugs, i.e. drugs with a lower bound of the 95% confidence interval (CI) of the odds ratio (OR) > 5 were pristinamycin (CI 26-infinity), ampicillin/amoxicillin (CI 10-infinity), quinolones (CI 8.5-infinity), (hydroxy)chloroquine (CI 8-infinity), anti-infective sulphonamides (CI 7.1-infinity), terbinafine (CI 7.1-infinity) and diltiazem (CI 5.0-infinity). No significant risk was found for infections and a personal or family history of psoriasis (CI 0.7-2.2). Conclusions Medications associated with AGEP differ from those associated with Stevens-Johnson syndrome or toxic epidermal necrolysis. Different timing patterns from drug intake to reaction onset were observed for different drugs. Infections, although possible triggers, played no prominent role in causing AGEP and there was no evidence that AGEP is a variant of pustular psoriasis.

Resume Introduction Les demandes de consultations en urgence semblent assez frequentes en cabinet de dermatologie liberale, cependant ni cette frequence ni les motifs de demande n’ont ete evalues. L’objectif principal de cette etude etait d’estimer cette demande de maniere qualitative et quantitative. L’objectif secondaire etait de chercher une association entre les demandes dont l’urgence etait justifiee a 48 heures et la symptomatologie invoquee par les malades afin d’etablir un score predictif de la validite de la demande. Methodes Quarante dermatologues repartis sur la France metropolitaine ont ete recrutes sur la base du volontariat. L’etude s’est deroulee pendant une semaine d’avril 2004, durant laquelle les dermatologues avaient allege leurs rendez-vous programmes de facon a recevoir tout malade demandant a etre vu en urgence. Les renseignements collectes concernaient : l’activite habituelle des dermatologues, les motifs d’appel, la symptomatologie, le diagnostic et l’appreciation par le dermatologue du degre d’urgence. L’analyse des donnees a ete univariee puis multivariee. Un score a pu etre realise a partir des coefficients des variables retenues dans le modele de regression logistique. Resultats Le nombre moyen de malades vus en urgence par chaque dermatologue participant a l’etude a double par rapport a une semaine normale. Durant la semaine d’etude, 613 malades ont demande un rendez-vous en urgence et la totalite des questionnaires a ete remplie pour 538 (88 p. 100) d’entre eux. Les diagnostics les plus frequemment poses par les dermatologues etaient : eczema, infections, dermatite atopique, mycose, naevus. Les dermatologues ont estime que la consultation etait justifiee dans les 48 heures pour un tiers des malades. En analyse multivariee, les facteurs significativement associes a une urgence justifiee etaient plus generaux que dermatologiques. Le score realise avait une sensibilite mediocre et aucun seuil ne permettait de bien classer plus de 77 p. 100 des demandes. Discussion La demande de rendez-vous en urgence est importante et consideree comme justifiee dans les 48 heures pour un tiers des malades. Les motifs et diagnostics sont proches de ceux observes dans les consultations d’urgence hospitaliere a l’urticaire pres. Les criteres associes a une urgence justifiee sont peu dermatologiques. Le score etabli a partir de ces criteres n’a pas ete suffisamment sensible pour permettre une selection telephonique fiable des malades.

Summary Background Few epidemiological studies assessing the prevalence of chronic dermatosis are available in France and most of these studies have used biased samples drawn from specific subpopulations. As several recent studies have mentioned that self-reported diagnosis either underestimates or overestimates disease prevalence, the validity of such data is questionable. Objectives To evaluate the agreement between self-reported and dermatologists’ diagnoses for five chronic dermatoses: acne, eczema, fungal infection, psoriasis and seborrhoeic dermatitis, and to analyse the factors associated with patients’ diagnosis knowledge. Patients/methods A cross-sectional study was conducted on the national day of skin tumour screening in April 2003. A self-administered questionnaire was completed by patients and a standard form was completed by dermatologists after clinical examination. A total of 4622 adults were examined by a dermatologist in 134 screening centres across France. The agreement between self-reported and dermatologists’ diagnoses was analysed using the kappa index. Characteristics of patients who were aware of their diagnosis were compared with those of patients who were not, by using multivariate logistic regression models. Results The demographic characteristics of the sample differed from those of the French population. The self-reported prevalence was significantly lower than the actual prevalence for all diseases except eczema. Underestimations ranging from 23% to 35% were observed in nearly all subgroups of our population. The agreement between self-reported and dermatologists’ diagnoses was low to moderate. Many cases of chronic skin diseases were diagnosed in patients who did not report them. Diagnosis knowledge was poorer in those above 54 years of age and better for patients treated for the condition and those with impairment of social life. Conclusions We observed a low agreement between self-reported and dermatologists’ diagnoses for five chronic diseases. Self-reports underestimated the actual prevalence of four of five common diseases.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but severe cutaneous adverse drug reactions, which can be caused by a certain number of specific drugs among which is carbamazepine, an antiepileptic agent. A very strong association of carbamazepine-induced SJS with HLA-B*1502 has recently been described in the Han Chinese population. Here in, we report preliminary results from a European study (RegiSCAR) of 12 carbamazepine-induced SJS/TEN cases (nine French and three German). Among these only four had a HLA-B*1502 allele. Remarkably, these four patients had an Asian ancestry, whereas the others did not as far as we have ascertained. This shows that although the HLA region may contain important genes for SJS, the HLA-B*1502 allele is not a universal marker for this disease and that ethnicity matters.

Resume Introduction La pemphigoide est une maladie bulleuse auto-immune dont la presentation clinique et la severite sont tres variables. Le role pathogene des anticorps anti-BPAG2 a ete recemment montre experimentalement. Le but de cette etude etait de rechercher une eventuelle relation entre la symptomatologie des malades atteints de pemphigoide et les antigenes reconnus par leurs auto-anticorps seriques. Malades et methodes Cent douze malades atteints de pemphigoide ont ete inclus dans cette etude prospective multicentrique. Les criteres d’inclusion etaient : 1) un diagnostic de pemphigoide etabli sur la presence de 3 des 4 criteres cliniques de pemphigoide, associe a un aspect histologique compatible et a une immunofluorescence directe positive, 2) un serum disponible pour une etude en immunotransfert. Les signes cliniques et biologiques etaient notes prospectivement sur des cahiers pre-imprimes. Les serums ont ete centralises et etudies en immunofluorescence indirecte et en immunotransfert sur extrait d’epiderme humain. Resultats L’analyse des signes cliniques en fonction des antigenes reconnus par les serums montrait que les malades dont le serum reconnaissait la proteine BPAG1 avaient plus frequemment un prurit, des bulles localisees sur les membres et une immunofluorescence indirecte plus souvent positive. Les malades dont le serum reconnaissait la proteine BPAG2 avaient plus frequemment des bulles localisees sur la tete et une immunofluorescence indirecte plus souvent negative. Les malades dont le serum etait negatif en immunotransfert avaient moins frequemment des plaques urticariennes et/ou eczematiformes, des bulles moins frequemment localisees sur la partie inferieure du tronc, l’abdomen et les membres inferieurs, une eosinophilie moins importante et une immunofluorescence indirecte plus souvent negative. Conclusions Les signes cliniques et biologiques les plus caracteristiques de pemphigoide sont trouves avec une plus grande frequence chez les malades dont le serum contient des anticorps diriges contre BPAG1.

SummaryBackground The histopathological features of drug rash with eosinophilia and systemic symptoms (DRESS) syndrome remain poorly characterized. Objectives To better characterize the histopathological features of DRESS syndrome, and define the phenotype of the effector cells in the skin and compare it with maculopapular rash (MPR). Methods We conducted a retrospective study on 50 skin biopsies from patients with DRESS syndrome (n = 36). Histopathological and immunophenotypical features were studied and compared with a series of MPRs (n = 20). Results Foci of interface dermatitis, involving cutaneous adnexae, were frequently seen in cases of DRESS. Eosinophils were seen in only 20% of cases and neutrophils in 42%. Eczematous (40%), interface dermatitis (74%), acute generalized exanthematic pustulosis-like (20%) and erythema multiforme-like (24%) patterns were observed. The association of two or three of these patterns in a single biopsy was significantly more frequent in cases of DRESS than in a series of nondrug-induced dermatoses (P

Summary Background Acute-stage specific bronchial epithelial detachment has been described in 27% of patients with Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). Objectives To assess the pulmonary function of patients with SJS/TEN after remission. Methods Analysis of pulmonary function tests (PFTs) performed during the usual follow-up of patients with SJS/TEN managed in a referral centre from April 2007 to January 2010. Results Of 58 patients admitted, 32 underwent PFTs (17 male, 15 female). The median time from the acute stage to PFTs was 3 months (interquartile range 1–18). Three patients had grade 2 dyspnoea. Eighteen patients (56%) had abnormal PFTs, including 13 patients (41%) with moderately altered diffusion capacity for carbon monoxide (DLCO) normalized by the alveolar volume (VA) (giving the ratio KCO, which equals DLCO/VA) and five patients with decreased total lung capacity. No airway obstruction was observed. Patients with decreased KCO had higher initial detached body surface area than others (30% vs. 10%, P = 0·006), as did those with decreased DLCO (25% vs. 10%; P = 0·054). There were correlations between detached body surface area and both KCO (r = −0·41, P = 0·026) and DLCO (r = −0·47, P = 0·011). Among 10 patients with decreased KCO on the first PFT, eight patients had a sustained decrease in KCO on a second PFT. Conclusions More than half of patients with SJS/TEN displayed abnormalities on PFTs, mainly diffusion impairment, which was associated with higher initial skin surface detachment. These abnormalities were mostly asymptomatic and remained stable over time.

Introduction Le syndrome de Stevens-Johnson et la necrolyse epidermique toxique (SJS/NET) mettent le pronostic vital et fonctionnel et s’accompagnent de sequelles organiques frequentes et sous-estimees. Le retentissement psychologique du SJS/NET a precedemment ete demontre avec une prevalence de 73 % de syndrome de stress post-traumatique (SDSPT) dans une serie retrospective. Une nouvelle evaluation prospective de la prevalence du SDSPT a ete proposee apres mise en place d’une strategie de prevention primaire. Materiel et methodes Nous avons conduit une etude prospective de juin 2009 a mars 2013 incluant l’ensemble des patients consecutifs pris en charge pour un SJS/NET, en capacite et non oppose a une evaluation sequentielle clinique et psychiatrique. Des entretiens semi-directifs et questionnaires (PDI, PDEQ, PTSD) etaient proposes a m0, m6, m12 permettant de reperer les repercussions psychologiques et en particulier l’existence ou non d’un SDSPT. Les donnees cliniques dermatologiques etaient colligees en parallele. L’analyse statistique univariee etait realisee a partir des tests de Mann-Whitney et de Chi 2 . Resultats vingt-neuf patients (20 femmes et 9 hommes) d’âge median 45 ans ± 15,7 (21–74) ont ete inclus. Le suivi a un an etait realise chez 26 patients. Seize (55,1 %) presentaient une detresse peri-traumatique, 25 (86,2 %) des reactions dissociatives peri-traumatiques. Un SDSPT etait objectivait chez 9 patients (31 %), correle a la presence d’une detresse peri-traumatique ( p = 0,01). La surface decollee-decollable mediane etait de 17 % (1–90) avec un SCORTEN median de 2 (0–5). Les complications dermatologiques en phase aigue comprenaient sepsis ( n = 10), reanimation ( n = 10), intubation ( n = 10). La severite de la maladie dermatologique (surface corporelle maximale decollee, SCORTEN, sepsis, intubation, reanimation), les antecedents psychiatriques n’etaient pas associes a la survenue du SDSPT. Discussion Le SJS/NET par sa soudainete et la violence de la confrontation a la mort qu’elle impose au patient et a ses proches, a une dimension d’evenement traumatique susceptible d’entrainer potentiellement des reactions post-traumatiques et des troubles anxieux ou de l’humeur particulierement invalidants. Leur prevalence a ete mesestimee face a l’urgence de la prise en charge organique. En comparaison de l’etude retrospective, nous objectivons une nette diminution du SDSPT dans le SJS/TEN. Ce resultat peut en partie etre explique par une strategie de prevention primaire faisant intervenir des la phase aigue et durant au moins une annee un suivi psychologique et psychiatrique. Conclusion Les manifestations de detresse psychique consecutives a l’hospitalisation pour un SJS/NET ne sont pas systematiques mais sont frequentes. Il semble necessaire d’organiser dans ce contexte une prise en charge psychologique precoce et prolongee.

Background: Lichen planus (LP) pemphigoides is defined by the concurrence of bullous pemphigoid (BP) and LP lesions. However, other autoimmune bullous skin diseases such as paraneoplastic pemphigus or bullous lupus erythematosus may also be associated with lichenoid eruptions. Objective: Our purpose was to describe 3 African patients with a similar form of lichenoid erythrodermic BP. Methods: Medical records of the 3 patients were reviewed for clinical, histologic, and biologic data. The ultrastructural localization of autoantibody deposits was studied by direct and indirect immunoelectron microscopy. The antigenic specificities recognized by the patients' sera were analyzed by immunoblotting on human epidermal extracts. Results: Three black African men had a severe lichenoid erythroderma associated with BP lesions involving the skin and mucosa. Histologic and direct immunofluorescence examination of skin and mucosal lesions were consistent with the diagnosis of BP associated with a lichenoid dermatitis. Immunoblot analysis of sera detected anti-BPAG1 and anti-BPAG2 antibodies and immunoelectron microscopy showed IgG deposits localized in the lamina lucida and the hemidesmosomes. The 3 patients were natives of an area near the Senegal River and had the common HLA-DR10 haplotype. Conclusion: The clinical and histologic features of these 3 patients were distinct from classic LP pemphigoides. Their common geographic origin and the presence of the HLA-DR10 haplotype suggest that these cases represent a particular type of lichenoid erythrodermic BP, which is probably determined by genetic factors.(J Am Acad Dermatol 1998;39:691-7.)

BackgroundCases of severe drug hypersensitivity, demonstrating a variable spectrum of cutaneous and systemic involvement, are reported under various names, especially drug reaction with eosinophilia and systemic symptoms (DRESS). Case definition and overlap with other severe cutaneous adverse reactions (SCAR) are debated.ObjectivesTo analyse the spectrum of signs and symptoms of DRESS and distribution of causative drugs in a large multicentre series.Patients and methodsRegiSCAR, a multinational registry of SCAR, prospectively enrolled 201 potential cases from 2003 to mid-2009. Using a standardized scoring system, 117 cases were validated as showing probable or definite DRESS.ResultsThe male/female ratio was 080; females were borderline significantly younger than males. Next to the ubiquitous exanthema, the main features were eosinophilia (95%), visceral involvement (91%), high fever (90%), atypical lymphocytes (67%), mild mucosal involvement (56%) and lymphadenopathy (54%). The reaction was protracted in all but two patients; two patients died during the acute phase. Drug causality was plausible in 88% of cases. Antiepileptic drugs were involved in 35%, allopurinol in 18%, antimicrobial sulfonamides and dapsone in 12% and other antibiotics in 11%. The median time interval after drug intake was 22days (interquartile range 17-31) for all drugs with (very) probable causality, with differences between drugs.ConclusionThis prospective observational study supports the hypothesis that DRESS is an original phenotype among SCAR in terms of clinical and biological characteristics, causative drugs, and time relation. The diversity of causative drugs was rather limited, and mortality was lower than that suggested by prior publications.

Summary Background Acute generalized exanthematous pustulosis (AGEP) is a severe cutaneous adverse reaction characterized by rash with sterile pustules, high fever and elevated circulating neutrophil counts. Objectives To investigate the frequency and clinical features of AGEP systemic involvement. Methods This retrospective study included all patients hospitalized in our department between 2000 and 2010 with a discharge diagnosis of AGEP. Patients had to fulfil the following criteria: (i) a specific EuroSCAR score > 4 and (ii) biological and radiological work-up available. Results Among the 58 patients enrolled, 10 had at least one systemic involvement: hepatic function test results were abnormal for seven; six had renal insufficiency; two developed acute respiratory distress, with one patient's bronchoalveolar lavage fluid containing many neutrophils but no microorganisms; one was agranulocytotic. Mean peripheral neutrophil counts and mean C-reactive protein levels were elevated significantly in patients with systemic involvement. Amoxicillin rechallenge and hospitalization duration were associated with systemic involvement. AGEP systemic involvement was observed in 17% of cases studied, including liver, kidney, bone-marrow and lung involvement. Outcomes were favourable after drug withdrawal, and symptomatic and topical steroid treatments. Conclusions The neutrophil count–systemic involvement association may suggest a role for neutrophils in AGEP systemic involvement. Physicians should be aware of the possibility of systemic involvement in AGEP and should actively look for signs of extracutaneous reactions.

SummaryBackground Linear IgA bullous dermatosis (LABD) is a rare autoimmune blistering skin disorder characterized by linear deposits of IgA along the dermoepidermal junction, visualized by direct immunofluorescence (DIF). It is usually spontaneous and drug induced. Objectives To compare the clinical and histological forms of LABD. Methods This retrospective single-centre cohort study concerned 28 patients diagnosed with LABD between 1 January 1995 and 31 December 2010. Imputability, determined according to the French imputability method (modified Begaud score) and Naranjo score, enabled classification into drug-induced and spontaneous LABD groups. Clinical and histological features were compared by blinded analysis of images and histological patterns. Results Sixteen patients had spontaneous LABD and 12 had drug-induced LABD. Nikolsky sign and large erosions were significantly more frequent in drug-induced than spontaneous LABD (P = 0·003 and P = 0·03, respectively), with no between-group differences for erythematous plaques, target or target-like lesions, string of pearls, location, mucosal involvement or histological features. Conclusions Drug-induced LABD was more severe than the spontaneous form, with lesions mimicking toxic epidermal necrolysis. Because LABD may be polymorphic and sometimes life threatening, DIF assay is recommended for all patients with Nikolsky sign and large erosions.

Resume Introduction Les infections cutanees a Mycobacterium fortuitum ( M. fortuitum ) sont rares, le plus souvent iatrogenes. Nous rapportons un cas avec atteinte cervicale survenue apres une intervention pour lifting. Observation Une femme de 65 ans, sans antecedent, avait eu un lifting facial chirurgical bilateral. Il s’etait complique d’une necrose cutanee traitee par cicatrisation dirigee a domicile. Six semaines plus tard, apparaissaient un nodule abcede sous-maxillaire gauche, draine chirurgicalement, puis d’autres nodules inflammatoires pre-auriculaires et cervicaux gauches, sans adenopathie, ni fievre. Les prelevements bacteriologiques isolaient un M. fortuitum . L’antibiotherapie, initialement probabiliste par clarithromycine, secondairement adaptee par amikacine et ciprofloxacine puis amikacine et imipeneme, pour une duree totale de 3 mois, permettait d’obtenir une guerison clinique. Discussion M. fortuitum est une mycobacterie a croissance rapide, ubiquitaire, responsable d’infections nosocomiales chez les sujets immunocompetents, notamment apres chirurgie plastique. La contamination survient en un site de rupture de la barriere cutanee, par contact avec un vecteur (eau, materiel chirurgical, antiseptique…). Le traitement, mal codifie, consiste en l’association d’un traitement chirurgical et d’une antibiotherapie pendant plusieurs mois.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are immunologically mediated, severe cutaneous adverse reactions involving cytotoxic T cells, natural killer cells and various mediators. In large studies, up to 15% of SJS/TEN occurred in patients with chronic corticosteroid use. It is unclear if this prior exposure to corticosteroids modified the disease course.To evaluate whether systemic corticosteroid usage prior to the onset of SJS/TEN modified the clinical course and outcome. If a disease-modifying effect is present, information from such an analysis may have implications on the therapeutic use of corticosteroids in SJS/TEN.This is a case-control study based on data collected in the EuroSCAR and RegiSCAR studies. Ninety-two cases of SJS/TEN with exposure to corticosteroids prior to the onset of disease, and 321 randomly selected SJS/TEN patients without prior exposure were included. Primary outcomes included progression of disease, disease severity and mortality. A secondary analysis of latency between the beginning of drug use and the onset of disease, based on exposure to a single high-risk drug, was also performed.On multivariate analysis, cases with prior exposure to corticosteroids had a longer progression of disease by 2·2 days [95% confidence interval (CI) 1·1-3·2]. The disease severity and mortality outcome were unaffected. In addition, there is evidence that corticosteroids delayed the onset of SJS/TEN in patients with exposure to high-risk drugs by 7·1 days (CI -0·2 to 14·5).The prior use of corticosteroids prolonged the period of disease progression without influencing the disease severity or mortality. In addition, when SJS/TEN is preceded by use of a single high-risk drug, the latency between the drug intake and the onset of SJS/TEN may also be increased. These findings suggest that corticosteroids have a mild impact on the course of SJS/TEN, and further studies are required to clarify any potential therapeutic effects.

Keratinocytes have recently been reported to contain creatine kinase (CK) of brain-type isoenzyme. The aim of this study was to investigate whether necrosis of keratinocytes induced raised CK levels in toxic epidermal necrolysis (TEN). The serum and blister fluid levels of creatine kinase and its isoenzymes [muscular-type (MM), brain-type (BB), myocardial-type (MB)] were measured in 40 patients with TEN, 10 patients with other bullous dermatoses, and in suction blisters in five controls. The mean serum CK was significantly higher in TEN patients than in patients with other bullous dermatoses (mean +/- SD: 480 +/- 535 U/l vs. 107 +/- 44 U/l, P < 0.05). The MM-isoenzyme was predominant (94%). A positive correlation was found between the level of the serum CK and the percentage of body surface area (BSA) involved (r = 0.49, P < 0.001). The mean blister CK was significantly higher in TEN patients than in patients with other bullous dermatoses or controls (mean +/- SD: 728 +/- 437 U/l vs. 310 +/- 244 U/l and 268 +/- 194 U/l, respectively, P < 0.02). The isoenzyme distribution of blister CK in TEN patients was: 76.8% MM, 18.1% MB and 5% BB. Although a significant part of blister CK comigrating with CK-MB, after preincubation with protein A-Sepharose, appeared to be CK-BB/IgG complex, the CK-BB fraction constituted less than 25% of blister CK. Therefore, the CK present in increased amounts in serum and blister fluid in TEN was not directly produced by keratinocytes.

Introduction Le syndrome de Stevens-Johnson (SJS) et la necrolyse epidermique toxique (NET) ont une mortalite globale de 22 %. Le but de ce travail etait d’evaluer l’interet de la ciclosporine prescrite de facon protocolisee dans un centre expert. Patients et methodes Une etude retrospective etait realisee incluant l’ensemble des patients consecutifs hospitalises dans le centre pour SJS-NET de janvier 2005 a mars 2014. La ciclosporine etait administree per os (3 mg/kg/j) en l’absence de sepsis, d’hypertension arterielle non-controlee ou d’insuffisance renale chronique. Une evaluation sequentielle clinique, biologique etait menee. L’analyse de la mortalite avec ou sans ciclosporine etait effectuee avec une courbe de Kaplan-Meier et un test de log-rank. Une analyse univariee des facteurs associes a la mortalite etait realisee. Enfin, un modele mixte etait utilise pour l’analyse de la survie et l’evolution du decollement avec ou sans ciclosporine. Resultats Cent trente patients consecutifs etaient inclus dont 67 femmes (51,9 %) d’âge moyen 47 ± 19,6 ans. Soixante-quinze (57,7 %) etaient traites par ciclosporine. La surface moyenne decollee-decollable a J0 etait de 13,7 ± 17,7 %. Le SCORTEN a J0 etait de 1,46 ± 1,17 (0–7). Vingt-trois patients etaient intubes. Neuf deces etaient observes (7 %) sans influence de la ciclosporine (RR : 0,75 [0,20–2,7]). Les facteurs associes a la mortalite comprenaient les antecedents de cancer (RR : 5 [1,2–20,1]), l’insuffisance hepatique (RR : 94,1 [7–709]), la survenue d’une insuffisance renale aigue (RR : 5,9 [1,3–26,2]), le recours a la ventilation mecanique (RR : 29,6 [3,6–241,1]). Le decollement moyen du groupe sans ciclosporine etait significativement inferieur a j0 (9,7 % vs 16,5 %). Le decollement tendait a augmenter dans le bras sans ciclosporine sans etre significatif. L’initiation de la reepithelialisation etait plus rapide sous ciclosporine (p = 0,049). Discussion Cette serie incluant l’ensemble des patients consecutifs pris en charge en dermatologie (80 %) et reanimation (20 %) montre un taux de mortalite global de 7 % sur les 10 dernieres annees. Les facteurs explicatifs autres que la ciclosporine devant etre pris en compte sont l’amelioration des soins symptomatiques et des defaillances d’organe et une admission plus precoce. Cette serie importante confirme la tendance a l’arret de la progression du decollement et le debut de reepithelialisation plus precoce dans le groupe ciclosporine. Ceci pourrait etre lie d’apres une publication recente a un effet des inhibiteurs de la calcineurine sur la migration cutanee de progeniteurs medullaires, favorisant ainsi la cicatrisation. Conclusion La prise en charge du SJS-TEN incluant l’amelioration des soins symptomatiques et des defaillances d’organe, la ciclosporine precoce, pourraient contribuer a une amelioration du taux de mortalite.

DRESS syndrome is a severe adverse drug reaction with visceral involvement. Its physiopathology includes immunological disorders associated with human herpes virus (HHV) reactivation. We report two cases of auto-immune thyroiditis occurring in the context of DRESS syndrome associated with HHV-6 reactivation.Case 1 : A 39-year-old woman presented DRESS syndrome with HHV-6 reactivation, cutaneous, lymph node, hepatic and renal disorders treated with systemic corticosteroids for 10 months. Following discontinuation of the corticosteroids, she developed Graves's disease, which was stabilized with carbimazole and a beta-blocker. CASE 2: A 31-year-old woman was hospitalized for DRESS syndrome with delayed HHV-6 reactivation and severe hepatic involvement. She was successfully treated by topical steroids. Six weeks later, she presented De Quervain thyroiditis associated with moderate relapsing DRESS, which were treated by sodium levothyroxine and topical steroids.There is currently debate about the implication of viral reactivation, in particular HHV-6, in chronic DRESS, relapse and development of auto-immune diseases. These observations highlight the potential risk of patients developing auto-immune diseases and underline the need for prolonged clinical and laboratory follow-up of patients with DRESS.

Background Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN)are acute mucocutaneous reactions associated with poor prognosis. The treatment is mainly symptomatic, based on supportive care. Until now, several curative treatments have been proposed without evidence of effectiveness.Objectives To evaluate the effect of ciclosporin on SJS and TEN after a short series had suggested a benefit.Methods We conducted an open, phase II trial to determine the safety and possible benefit of ciclosporin. Among the 45 consecutive patients admitted for SJS /TEN from March 2005 to September 2007, 29 fulfilled inclusion criteria. Ciclosporin was administered orally (3 mg kg)1 daily for 10 days) and tapered over a month. Clinical and biological evaluations were performed sequentially. Predicted death rate was estimated with a validated prognostic score (SCORTEN).Results Twenty-nine patients were included at a mean +/- SD of 2.8 +/- 1.8 days after onset. The final diagnosis was SJS (n = 10), SJS ⁄TEN overlap (n = 12) and TEN(n = 7). One month of treatment was completed in 26. Ciclosporin was stopped after more than 10 days in three cases for side-effects including posterior leucoencephalopathy (n = 1), neutropenia (n = 1) and nosocomial pneumopathy(n = 1). Ciclosporin dosage was tapered earlier than scheduled in two cases for alteration in renal function. The prognostic score predicted 2.75 deaths; none occurred (P = 0.1). Mean epidermal detachment remained stable in 18 of 29 cases (62%). The mean ± SD hospital stay was 16.2 +/- 9.1 days.Conclusions Both the death rate and the progression of detachment seemed lower than expected, suggesting a possible usefulness of ciclosporin in SJS and TEN that needs to be confirmed.

Potent topical corticosteroids (TCS), such as clobetasol propionate are more efficacious than systemic corticosteroids in the treatment of bullous pemphigoid (BP) and in reducing the rate of systemic infectious complications. However, TCS can have cutaneous side effects, such as atrophy and purpura. The risk of cutaneous infections due to TCS in BP is known but has never been studied, despite prolonged use of high doses. Since we noted three cases of fatal necrotizing fasciitis (NF) in patients treated with TCS for BP over a one-year period in our institution, we decided to analyse the frequency of cutaneous infections in all BP patients hospitalized in our department.In this retrospective single-centre study, all files of patients presenting BP treated with TCS and hospitalized between April 2008 and April 2009 were reviewed. When the clinical file indicated a cutaneous infectious problem, bacteriological data were requested from the bacteriology laboratory. For each patient, clinical data, history, ongoing treatment, type of cutaneous infectious complication, general symptoms and details of outcome were collected.In the 30 files studied, we found ten cutaneous infections in nine patients: minor complication (three cases of impetiginisation), moderate complications (two erysipelas, one lymphangitis with sepsis, one flexor tendon phlegmon with cutaneous fistula), and severe complications with a fatal course (three NF, one of which had involved erysipelas of favourable outcome a few months earlier). These cutaneous complications occurred after various treatment times (ten days to two years) and various dosages of TCS (two-three tubes/day to two tubes every two days). Three of the nine patients with cutaneous infections had diabetes, in particular two of the three patients with FN. In contrast, four of the 21 patients without cutaneous complications had diabetes. Patients with cutaneous infections did not have more extensive BP or receive more TCS than the others. In two of three patients with NF, an immunosuppressant drug (methotrexate or mycophenolate mofetil) had been recently initiated (inferior to one month). The offending organism was Staphylococcus aureus in seven cases (methicillin-resistant in three cases) and Streptococcus A in five cases (three NF, one lymphangitis and one impetiginisation). The outcome was fatal in the three cases of NF but was favourable with local and/or systemic antibiotic therapy in the remaining cases.In this study, we noted cutaneous superinfection in nine of 30 (30%) patients receiving topical corticosteroids for bullous pemphigoid, among which were three cases of fatal NF due to streptococcus A (10%). The infectious risks associated with TCS must not be neglected, particularly since treated patients are old and fragile, and frequently have multiple well-known risk factors for NF (e.g. extensive lesions, diabetes, etc.). In the event of signs of cutaneous superinfection, especially in cases of diabetes and prolonged TCS treatment, bacteriological analysis should be conducted. Adequate treatment should be initiated without delay, especially in cases of beta-haemolytic streptococcal infection.

To investigate whether propensity score (ps) methods could reasonably be applied to estimate the treatment effect on mortality, based on a comparatively small sample of patients with severe cutaneous adverse reactions (SCAR) and who come from different countries where physicians prefer different treatment schemes.Ps methods were applied to cope with confounding due to non-randomized treatment assignment for the analysis of the treatment data obtained in the case-control study EuroSCAR. For the study's purpose, the analysis focused on the comparison of the treatments: corticosteroids (STER) and supportive care only (SUPP).206 French and German patients were treated either with SUPP or STER. Imbalances between treatment groups as well as between the countries were recognized. Concerning the balance between the treatment groups no ps model for the full cohort was satisfying. In addition, the inclusion of a variable for patient's country led to a separation of the patients by country. Thus, we developed ps models for each country separately and estimated the treatment effects (France: odds ratio (OR) 0.52, 95% confidence interval (CI) 0.09-3.10, Germany: OR 0.23, CI 0.06-0.92, Overall: OR 0.33 CI 0.11-1.04).The application of the ps methods was successful and provided valuable information. We could confirm the findings of the original analysis which was based on standard logistic regression, especially concerning the necessity of a country-specific analysis. The observed country differences in the estimated treatment effects were less pronounced and thus seemed to be more reasonable than those of the past analysis.

There have been several recent advances in the management of auto-immune blistering diseases. The aim of this review was to provide an update, based on ''expert'' interpretation of published evidences on the treatments of pemphigoid and pemphigus. A literature search (Pubmed) of all randomized controlled trials that had not been included in two reviews published by the Cochrane Skin Group in 2005 on pemphigoid and 2009 on pemphigus have been performed. Recent series of pemphigus cases treated with anti-CD20 antibodies have also been reviewed. Concerning pemphigoid there is good evidence that high dose topical clobetasol propionate should be the first line treatment, and that further studies should focus on the finding of simple and well tolerated maintenance therapy. For pemphigus, after decades of inconclusive trials, there is now some evidence of a moderate efficacy of azathioprine, cyclophosphamide, mycophenolate in addition to corticosteroids and may be of intravenous immunoglobulins. The practical impact of such evidence is strongly challenged by the possible superiority of anti-CD20 monoclonal antibodies that may emerge as the most efficient treatment of pemphigus. Because of concerns on long-term safety, multicenter trials of these ''biological'' treatments should be undertaken urgently.

Psoas abscess is a rare clinical entity that occurs chiefly after intra-abdominal or retroperitoneal infection. We report two cases of psoas abscesses caused by group A beta-haemolytic streptococcal infection having a cutaneous portal of entry.The first patient, a 50-year-old man, was feverish and had ulcerative and necrotic cutaneous lesions evocative of ecthyma that were progressing for three months and were recently associated with a painful mass in the left iliac fossa, leading to difficulties in walking. The second patient, a 35-year-old woman with a medical history of intravenous drug addiction, was admitted to intensive care for sepsis syndrome following group A beta-haemolytic streptococcal infection with a cutaneous portal of entry (swelling on left lower limb). She remained unaccountably subfebrile 10 days after the start of antibiotic therapy with amoxicillin. Abdominal CAT scans for each patient confirmed the diagnosis of left psoas abscess. For the first patient, the same group A beta-haemolytic streptococcus was isolated in drainage fluid and at the cutaneous injury site. The outcome was favourable in both cases following extensive intravenous antibiotic therapy (amoxicillin) combined with percutaneous drainage (in the first case).Psoas abscess can occur after locoregional infection and the portals of entry are usually gastro-intestinal, musculoskeletal or genitourinary, with many organisms capable of causing such secondary abscesses. Psoas abscess can also be a primary clinical event. Staphylococcus aureus is the most common causative organism. The presented cases comprised secondary psoas abscesses with a cutaneous portal of entry. Since the complete set of three evocative symptoms (prolonged fever, pain and psoitis) is frequently seen late, diagnosis must be made on the basis of prolonged infectious state or unaccountable feverish abdominal pain. Diagnosis is based on abdominal CAT scan and treatment involves the use of appropriate antibiotics as well as percutaneous or surgical drainage of the abscess. The mortality rate in this patient population remains high with survival being dependent on prompt initiation of therapy.

Although often clinically suspected, infectious endocarditis (IE) is frequently difficult to diagnose with certainty. Although the dermatological signs of endocarditis can vary, they must be routinely sought where there is a suspicion of IE. The aim of this study was to reveal the diversity of clinical manifestations of IE in a dermatology unit.This retrospective study was conducted between May 2006 and May 2007 and included all patients hospitalized in the dermatology unit in whom an IE was diagnosed according to the modified Duke criteria.Seven patients were included with a median age of 61 years. The reasons for hospital admission were: chronic ulcers (n=1), Sezary's syndrome (n=1), atopic dermatitis (n=1), epidermolysis bullosa acquisita (n=1) and purpura (n=1). Specific dermatological manifestations of IE included necrotic lesions on the lower limbs (n=2), purpura (n=5) and splinter haemorrhages (n=1). Blood cultures were positive in 3 cases (MSSA=2, MRSA=1). One patient had serological evidence of Coxiella burnetti IE. Cutaneous sources of IE were found in 6 cases, including acute dermohypodermitis or chronic dermatosis (3), peripheral venous catheter (n=2) and haemodialysis (n=1). Transthoracic echocardiography was negative in 6 patients, whereas transoesophageal echocardiography performed in 6 patients confirmed the diagnosis in 5 cases. The mean time to diagnosis was 21 days. Among these patients, 5 died after a mean period of 78 days.Diagnosing IE remains a clinical challenge and must be routinely considered in the presence of unusual dermatological findings such as purpura or distal necrosis, but also in patients with partially or poorly controlled chronic dermatosis, which comprise an underestimated potential source of IE. Physicians treating such patients must consider the risk of IE, especially in the event of chronic dermatosis or of an invasive cutaneous procedure involving affected skin.

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).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.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.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.