E. Bourdon-Lanoy

Multicentre consensus recommendations for skin care in inherited epidermolysis bullosa

BackgroundInherited epidermolysis bullosa (EB) comprises a highly heterogeneous group of rare diseases characterized by fragility and blistering of skin and mucous membranes. Clinical features combined with immunofluorescence antigen mapping and/or electron microscopy examination of a skin biopsy allow to define the EB type and subtype. Molecular diagnosis is nowadays feasible in all EB subtypes and required for prenatal diagnosis. The extent of skin and mucosal lesions varies greatly depending on EB subtype and patient age. In the more severe EB subtypes lifelong generalized blistering, chronic ulcerations and scarring sequelae lead to multiorgan involvement, major morbidity and life-threatening complications. In the absence of a cure, patient management remains based on preventive measures, together with symptomatic treatment of cutaneous and extracutaneous manifestations and complications. The rarity and complexity of EB challenge its appropriate care. Thus, the aim of the present study has been to generate multicentre, multidisciplinary recommendations on global skin care addressed to physicians, nurses and other health professionals dealing with EB, both in centres of expertise and primary care setting.MethodsAlmost no controlled trials for EB treatment have been performed to date. For this reason, recommendations were prepared by a multidisciplinary team of experts from different European EB centres based on available literature and expert opinion. They have been subsequently revised by a panel of external experts, using an online-modified Delphi method to generate consensus.ResultsRecommendations are reported according to the age of the patients. The major topics treated comprise the multidisciplinary approach to EB patients, global skin care including wound care, management of itching and pain, and early diagnosis of squamous cell carcinoma. Aspects of therapeutic patient education, care of disease burden and continuity of care are also developed.ConclusionThe recommendations are expected to be useful for daily global care of EB patients, in particular in the community setting. An optimal management of patients is also a prerequisite to allow them to benefit from the specific molecular and cell-based treatments currently under development.

Early skin biopsy is helpful for the diagnosis and management of neonatal and infantile erythrodermas

Background: Erythrodermas are often life‐threatening conditions in infants. Determination of the underlying cause is crucial.

Pemphigoïde du sujet jeune: Étude rétrospective de 74 cas

INTRODUCTION Bullous pemphigoid usually affects elderly people. Only a few isolated cases among people younger than 65 years have been reported. OBJECTIVES Describe the clinical and biological characteristics of patients younger than 60 years suffering from bullous pemphigoid, compare them with the usual characteristics known among elderly people and search for potential pathological associations. PATIENTS AND METHODS Retrospective, national, multicenter study. Clinical, biological and histological characteristics were recorded with a standardised questionnaire as well as treatments and associated pathologies. RESULTS Seventy-four cases of bullous pemphigoid diagnosed between June 1970 and March 2002 were analyzed. Mean age at the beginning of the disease was 46 +/- 11.6 years. Further explorations by indirect immunofluorescence of separated skin and/or immuno-electron microscopy and/or immunoblotting were performed for 42 patients (56.8 p. 100). Clinical characteristics among this restricted population were comparable to those found among the 32 other cases. Compared to usual data on bullous pemphigoid in elderly people, we observed a greater proportion of extensive form of disease (75 p. 100), a more frequent head and neck involvement (39.2 p. 100) and an overexpression of anti-BP180 autoantibodies (48 p. 100). Neoplasm was notified for 7 patients (9.5 p. 100), 18 (24.3 p. 100) suffered from a pathology of the basement membrane zone (6 psoriasis, 6 atopic dermatitis and 6 lichen) and 13 from neurological disease, among which 4 were bedridden. Fourty-six patients (62.2 p. 100) received drugs for the long term (mean 2.12 +/- 2.43), 4 patients were treated by PUVAtherapy and 2 by radiotherapy. DISCUSSION Our results suggest that bullous pemphigoid among young people is more severe and more active than the usual form in the elderly. This particular form could be the result of a higher expression of anti-BP180 autoantibodies, which are considered as a marker of poor prognosis in this disease. We also found a high frequency of pathological associations and physical treatment, all responsible for damage to the basement membrane zone, which can involve auto-immunization against hemidesmosome components.Resume Introduction La pemphigoide touche classiquement les sujets âges. Seules des observations ponctuelles ont ete decrites chez les sujets avant 65 ans. Objectifs de l’etude Decrire les caracteristiques cliniques et biologiques d’une serie de malades âges de moins de 60 ans atteints de pemphigoide, de les comparer aux donnees des pemphigoides du sujet âge et de rechercher d’eventuelles associations pathologiques. Malades et methodes Il s’agissait d’une etude retrospective, nationale, multicentrique. Les caracteristiques cliniques, biologiques et histologiques, les donnees d’immunofluorescence, ainsi que les traitements et les pathologies associees ont ete recueillis grâce a un questionnaire standardise. Resultats Soixante-quatorze cas de pemphigoide chez des malades âges de moins de 60 ans, diagnostiques entre juin 1970 et mars 2002 dans les hopitaux participants, ont ete analyses. L’âge moyen de debut de la maladie etait de 46 ± 11,6 ans. Des explorations complementaires par immunofluorescence indirecte sur peau clivee et/ou par immunomicroscopie electronique et/ou par immunoblot ont ete realisees chez 42 malades (56,8 p. 100) dont les caracteristiques cliniques de pemphigoide etaient superposables a celles des 32 autres malades. Par rapport aux pemphigoides des sujets âges, on observait une plus forte proportion de formes multibulleuses (75 p. 100) avec une atteinte plus frequente de la tete et du cou (39,2 p. 100), et une plus grande frequence d’anticorps anti-BP180 (48 p. 100). Une neoplasie etait notee chez 7 malades (9,5 p. 100), 18 (24,3 p. 100) souffraient d’une autre dermatose (6 psoriasis, 6 dermatites atopiques et 6 lichens) et 13 malades (17,6 p. 100) d’une maladie neurologique dont 4 avec grabatisation. Quarante-six malades (62,2 p. 100) prenaient un traitement au long cours avec en moyenne 2,12 ± 2,43 medicaments, 4 malades avaient ete traites par PUVAtherapie et 2 par radiotherapie. Commentaires Ces donnees suggerent que la pemphigoide du sujet jeune est une maladie plus severe et plus active que la forme classique du sujet âge. Cette expression clinique particuliere pourrait etre la consequence de la plus forte prevalence d’anticorps anti PB-180. Ces anticorps sont consideres comme des marqueurs de mauvais pronostic et correles a l’activite chronique de la pemphigoide. On note aussi une frequence elevee d’associations pathologiques ou de traitements physiques pouvant generer des alterations de la jonction dermo-epidermique et favoriser l’auto-immunisation contre des constituants des hemidesmosomes.

Estimation de la fréquence des atteintes plantaires à dermatophytes: Étude rétrospective 2002-2003

BACKGROUND Plantar dermatophytosis frequently goes unnoticed and can cause relapse or re-infestation at other sites. The purpose of this study was to evaluate the incidence of plantar dermatophytosis in association with onyxis and intertrigo involving dermatophytes. PATIENTS AND METHODS This was a retrospective study in patients seen at mycology consultations between January 2002 and December 2003 and for whom culture revealed dermatophytes on the soles, interdigital spaces and/or toe nails. Gender, age and culture data were record from the laboratory workbooks. RESULTS 716 patients were included, giving 1291 samples. The sex ratio M/F was 1.5 with a mean age of 48 years. Samples of toe nail were obtained from 591 patients, with plantar samples from 433 patients and intertrigo samples from 267 patients. Plantar dermatophytosis was seen in 66.6% of patients with interdigital-plantar signs, in 75.1% of those with ungual involvement and in 73.9% of cases involving both. T. rubrum was the most frequently isolated dermatophyte. DISCUSSION Combine involvement of the sole, nail and/or interdigital space was seen in more than 2/3 of cases. Despite the retrospective nature of our study and the evident bias, our results suggest that plantar dermatophytosis is common and should be sought. The sensitivity and specificity of clinical screening methods merit investigation in a prospective study.

Oral epigallocatechin-3-gallate for treatment of dystrophic epidermolysis bullosa: a multicentre, randomized, crossover, double-blind, placebo-controlled clinical trial

Recessive dystrophic epidermolysis bullosa (RDEB) is a rare genodermatosis with severe blistering. No curative treatment is available. Scientific data indicated that epigallocatechin-3-gallate (EGCG), a green tea extract, might improve the phenotype of RDEB patients. In a multicentre, randomized, crossover, double-blind, placebo-controlled clinical trial, we evaluated a 4-month oral EGCG treatment regimen in 17 RDEB patients. We found that EGCG treatment was not more effective than placebo in modified intention to treat and per protocol analysis (n = 16; p = 0.78 and n = 10; p = 1 respectively). Tolerance was good. Specific organizational and technical difficulties of controlled randomized double-blind trials in EB patients are discussed.Trial registrationUS National Institutes of Health Clinical Trial Register (NCT00951964).

Demandes de rendez-vous en urgence en dermatologie libérale : ETude Urgences en DErmatologie libérale (ET.U.DE)

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.