Search

In recent years, significant progress has been made in the understanding of the pathophysiological mechanisms of acne and the role of Propionibacterium acnes. With this review, the authors aim to provide an update on the current understanding of the role of P. acnes in the development of acne lesions and analysing the potential implications for future treatments. A total of 188 articles published between January 1980 and March 2013 were searched using key words such as acne, P. acnes, microbiology, Corynebacterium acnes, acne vulgaris, pathogenesis, antibiotic, vaccination and a combination of those key words. From those articles, 77 were analysed in depth. Recent data confirm that P. acnes has a strong proinflammatory activity and targets molecules involved in the innate cutaneous immunity, keratinocytes and sebaceous glands of the pilosebaceous follicle and leads to the development of comedones. Furthermore, the profile of its different strains may differ between healthy subjects and acne patients. The better understanding of the role of P. acnes may allow for new perspectives in the treatment of acne. Novel therapies should target molecules implicated in the activation of innate immunity, including toll-like receptors, protease-activated receptors and topical antimicrobial peptides; the latter may be an alternative to topical antibiotics and thus a solution for limiting bacterial resistance induced by topical macrolides. Vaccines may also be promising. However, the most appropriate candidate remains to be selected.

Background and objective Many acne grading methods exist; however, there is no agreed-upon standard. Our objective was to create and validate a reproducible acne assessment scale for rating the severity of juvenile facial acne suitable for use in France and Europe. Methods The scale we created described the different types of acne lesions in a manner similar to global assessment scales used in clinical trials. The scale was then validated by seven expert dermatologists in the field of acne [the Global Evaluation Acne (GEA) group] first on 34 photographic cases of Caucasian acne patients and second by clinical examination of 22 acne patients. Results There was good agreement in Investigators’ assessments of acne both on photographs and patients (R = 0.8057; P

Resume Introduction La pustulose exanthematique aigue generalisee (PEAG) est une toxidermie dans la majorite des cas. De nombreux medicaments systemiques en sont responsables, alors que ce tableau est beaucoup plus exceptionnellement du a un medicament topique. Nous rapportons un cas de PEAG apres application d’Algipan®. Observation Un homme de 51 ans etait adresse pour une eruption cutanee febrile evoluant depuis 72 heures. Il avait comme seul antecedent des lombalgies et ne prenait aucun traitement au long cours. L’eruption, initialement localisee a la main gauche et au dos, s’etait rapidement etendue a l’ensemble du tegument, avec de multiples pustules non folliculaires sur un erytheme cuisant diffus. Aucun traitement systemique n’etait signale dans les jours precedents l’eruption. Cependant, on apprenait qu’il s’etait applique trois jours auparavant avec la main gauche du baume Algipan® sur le rachis lombaire. L’ensemble du tableau clinique evoquait une PEAG induite par l’Algipan®. L’evolution clinique etait rapidement favorable. Les tests epicutanes effectues trois mois plus tard montraient une positivite avec le produit entier, confirmant le diagnostic. Discussion La PEAG est une toxidermie dans plus de 90 % des cas, avec une implication preponderante des antibiotiques, notamment β-lactamines et macrolides. L’Algipan® est un traitement local contenant de la mephenesine, utilise comme antalgique et anti-inflammatoire dans les douleurs musculaires de l’adulte. Il n’est pas, a ce jour, rapporte de toxidermie grave a ce traitement. Les cas de PEAG induites par des topiques sont exceptionnels et celui que nous presentons est le premier impliquant la mephenesine. Il suggere d’ajouter ce topique a la liste des medicaments responsables de PEAG.

Background Acne is a highly heterogeneous disorder with respect to its regional involvement, demographics, lesion types and response to treatment. Homogeneous patient groups must therefore be created before measuring the severity of acne and interpreting the results of therapeutic trials. The individualization of ‘juvenile acne of the face’ is a first step in this direction. Methods Based on textbooks, major reviews of acne and a ′Medline′ literature search, we identified four key points to delineate sub groups of acne patients. Result ‘Juvenile acne of the face’ is defined by four main clinical criteria: (1) age (from puberty to age 25 years), (2) site (face), (3) lesion type (polymorphous), (4) absence of a causal factor. Other subgroups of ‘true’ acne are listed with their distinctive characteristics. Non-acne follicular diseases (i.e. without sebum retention) are listed. Discussion We chose ‘juvenile acne of the face’ to define the first homogeneous group of patients because it is the most frequent form of acne and the target of most acne drugs. It has a sufficiently uniform prognosis to devise a functional and reproducible severity rating scale with the aim of facilitating the evaluation and comparison of therapeutic methods. Conclusion The next step in our work will be to elaborate severity scales which are dedicated to a single type of acne.

Resume Introduction La ciclosporine est un des principaux agents immunosuppresseurs employes dans la prevention et le traitement du rejet de greffe d’organe, et a moindres doses dans le traitement d’affections dysimmunitaires. De nombreux effets indesirables cutaneomuqueux ont ete rapportes avec cette molecule, principalement chez les transplantes : hypertrichose, hyperplasie gingivale, infections cutanees, en particulier, virales. Nous rapportons ici une toxicite folliculaire inhabituelle. Observation Un homme de 22 ans atteint de mucoviscidose recevait une greffe bipulmonaire en janvier 2005. Apres six semaines de traitement immunosuppresseur, survenait une eruption subaigue micropapuleuse folliculaire, peu prurigineuse, etendue au tronc, aux faces d’extension des membres et au visage. Les donnees histologiques et microbiologiques permettaient de retenir le diagnostic de toxidermie folliculotrope, imputee a la ciclosporine. Une regression complete de l’eruption etait observee lors du remplacement de cette molecule par le tacrolimus. Discussion Trois observations comparables ont ete rapportees anterieurement dans la litterature, avec des alterations folliculaires histologiques caracteristiques, distinctes de celles observees pour l’hypertrichose ou les keratoses pilaires. Cet effet indesirable rare peut s’expliquer par l’action directe de la ciclosporine sur l’unite pilosebacee, avec prolongation de la phase anagene du cycle pilaire et a concentrations tissulaires superieures, par un effet toxique entrainant une dystrophie de l’appareil folliculaire. Habituellement observee apres plusieurs mois de traitement par ciclosporine, cette toxidermie est survenue avec un delai plus court chez ce malade, probablement du fait de taux plasmatiques parfois excessifs.

Resume Introduction Le retentissement psychologique de l’acne est souvent independant de l’intensite des lesions d’acne. Ainsi, certaines echelles ont ete construites et validees dans le but de quantifier la severite de l’acne telle que l’Echelle de Cotation des Lesions d’Acne (ECLA) alors que d’autres ont pour objectif d’evaluer la qualite de vie comme le questionnaire Cardiff Acne Disability Index (CADI) recemment valide en francais. L’objectif de ce travail a ete d’utiliser ces deux echelles chez un meme patient acneique afin de determiner s’il existait une correlation ou non entre ces deux echelles specifiques de l’acne et de determiner les groupes de malades influences par l’acne. Methodes Cent vingt-huit patients acneiques (21 ± 6,8 ans) vus par sept dermatologues ont ete inclus dans cette etude. La severite de l’acne a ete evaluee a l’aide de la grille ECLA, les sept dermatologues ayant ete formes a cette echelle. Par ailleurs, les patients remplissaient le questionnaire de qualite de vie CADI apres la consultation. Chaque facteur de l’echelle ECLA a ete croise avec chaque item du questionnaire CADI a l’aide d’une analyse de variance. Resultats Il n’existait pas de correlation entre les scores globaux des deux echelles ECLA et CADI (r2 = 0.0242). Cependant une correlation positive etait observee entre le score global CADI et les facteurs F1 et F3 de la grille ECLA (p = 0,0085 et p = 0,0373 respectivement). Inversement, le score global de l’echelle ECLA etait significativement correle a l’item 5 du questionnaire CADI c’est-a-dire la perception de l’acne (p = 0,0035). Conclusion L’acne a un impact sur la composante psychologique du patient meme dans des formes minimes. La grille ECLA associee au CADI apparaissent deux outils interessants et complementaires pour le dermatologue dans sa prise en charge complete du patient acneique.

Background: Acne can have important psychological repercussions. Objectives: We studied adolescents’ perceptions of acne, in a nonmedical context. Methods: Between November 2004 and January 2005, a questionnaire was presented to adolescents calling a youth helpline. It focused on perceptions of acne and, among callers with personal experience of acne, its severity and treatment. Callers were divided into those with acne, those having had acne and those never having had acne. Results: The questionnaire was completed by 1,566 callers. It showed that 51% of respondents with acne did not seek medical help; acne has as big a psychological impact as some far severer diseases; many respondents with no history of acne said they were very worried that they would develop this skin disorder. Conclusions: This study underlines the major psychological impact of acne. Young people need to be informed that acne can be treated.

Resume Introduction. – Le syndrome des ecchymoses douloureuses decrit par Gardner et Diamond en 1955 est caracterise par l'apparition spontanee d'ecchymoses douloureuses, sans anomalies biologiques associees, chez des jeunes femmes au profil psychologique pathologique. Exegese. – Nous rapportons trois observations de jeunes filles presentant un syndrome des ecchymoses douloureuses. Dans un cas, la mise en place d'une psychotherapie a entraine une nette amelioration des manifestations cutanees et articulaires et dans un autre cas, la placebotherapie a permis la disparition de la symptomatologie de facon prolongee. Conclusion. – Plusieurs hypotheses physiopathologiques sont proposees dans le syndrome de Gardner et Diamond. Cependant, les observations rapportees dans la litterature plaident en faveur d'une origine psychogene. C'est une prise en charge globale, a la fois psychologique et somatique, qui doit etre proposee a ces malades.

Resume Introduction Le syndrome de Wells est une dermatose reactionnelle a eosinophiles, surtout decrite en association avec des infections ou apres piqures d’insectes mais rarement au cours d’une hemopathie. Observation Une femme de 32 ans avait des placards papulo-nodulaires inflammatoires des flancs, d’apparition brutale evoluant par poussees spontanement resolutives. L’histologie montrait un infiltrat dermique riche en polynucleaires eosinophiles compatible avec un syndrome de Wells. L’examen revelait une franche hepatosplenomegalie associee a une polyglobulie vraie et a un syndrome myeloproliferatif. Le diagnostic de maladie de Vaquez etait retenu. Discussion Le syndrome de Wells est une entite anatomoclinique aux presentations polymorphes, non specifiques et variables selon l’âge des lesions. Dermatose reactionnelle a divers stimulus, elle peut reveler, entre autres, des hemopathies sous jacentes. Les manifestations cutanees precedent souvent le diagnostic hematologique mais peuvent etre meconnues. Dans la litterature, seuls deux cas d’association a une maladie de Vaquez ont ete rapportes. Notre observation, originale par le jeune âge de la malade, illustre cette manifestation cutanee revelatrice inhabituelle de la maladie de Vaquez.

Resume Introduction A cote des discrets depots infracliniques de mucine observes au cours de nombreuses dermatoses inflammatoires, des mucinoses dermiques, cliniquement visibles, peuvent parfois s’associer a des collagenoses. Il s’agit en premier lieu de lupus et beaucoup plus rarement de sclerodermies. Dans ce cas, la confusion avec un scleromyxœdeme ne doit pas etre faite. Observation Une femme de 51 ans avait des lesions erythemateuses infiltrees en bandes des faces internes des cuisses et des bras associees a des myalgies, des arthralgies et a des doigts boudines. Initialement le diagnostic de connectivite mixte etait evoque. Rapidement, le tableau se completait par une sclerodactylie, des telangiectasies, un syndrome de Raynaud et une atteinte œsophagienne permettant de porter le diagnostic de sclerodermie systemique de type CREST. La biopsie cutanee des lesions erythemateuses en bandes montrait une mucinose dermique. Un traitement par hydroxychloroquine permettait a la fois une regression des lesions de mucinose et une stabilisation de la sclerodermie qui restait peu evolutive apres 4 ans de recul. Commentaires Les mucinoses dermiques peuvent accompagner des lupus erythemateux, plus rarement des dermatomyosites, et exceptionnellement des sclerodermies. La presentation clinique est variable avec de larges plaques infiltrees, des lesions reticulees ou papulo-nodulaires. En revanche, des lesions en bandes telles que celles observees chez cette malade n’ont pas ete rapportees jusqu’ici. L’association d’une mucinose dermique localisee a une sclerodermie ne doit pas amener a porter a tort le diagnostic de scleromyxœdeme dont le pronostic est bien different. La survenue d’une mucinose au cours d’une collagenose pourrait etre liee a une synthese accrue de mucine par les fibroblastes sous l’influence de cytokines inflammatoires augmentees dans ce contexte.

The monoclonality of the T cell receptor gamma-chain gene was analyzed by polymerase chain reaction in skin and blood specimens of 85 patients with cutaneous T cell lymphomas including 67 mycosis fungoides, seven Sézary syndromes, and 11 CD30- nonepidermotropic cutaneous T cell lymphomas. A cutaneous T cell clone was detected in 69% of mycosis fungoides and 100% of Sézary syndromes. This frequency varied according to the clinical stage: 57% in early stages (Ia-IIa) to 96% in advanced stages (IIb-IV, Sézary syndrome). A peripheral blood T cell clone was detected in 42% of early stages and in 74% of late stages but was identical to the cutaneous one in 15% and in 63%, respectively. A significant association between initial clinical stage and T cell monoclonality was observed. In nonepidermotropic cutaneous T cell lymphomas, T cell monoclonality was detected in 55% of skin and 36% of blood samples. Univariate and multivariate analyses showed that, besides the initial clinical stage, an identical cutaneous and blood T cell clone was an independent prognostic factor for disease progression of mycosis fungoides/Sézary syndrome (hazard ratio 3.4, 95% confidence interval 1.4-9.9). Parallel polymerase chain reaction study of skin and blood specimens may therefore provide an initial prognostic marker that could help to monitor therapeutic strategies. A fully prospective study, with simultaneous therapeutic trials, needs to be done to confirm our findings and to include treatment variables in the statistical analysis.

Background: In addition to tetracyclines, zinc may constitute an alternative treatment in inflammatory lesions of acne. Objective: To evaluate the place of zinc gluconate in relation to antibiotics in the treatment of acne vulgaris. Methods: Zinc was compared to minocycline in a multicenter randomized double-blind trial. 332 patients received either 30 mg elemental zinc or 100 mg minocycline over 3 months. The primary endpoint was defined as the percentage of the clinical success rate on day 90 (i.e. more than 2/3 decrease in inflammatory lesions, i.e. papules and pustules). Results: This clinical success rate was 31.2% for zinc and 63.4% for minocycline. Minocycline nevertheless showed a 9% superiority in action at 1 month and one of 17% at 3 months, with respect to the mean change in lesion count. Regarding safety, the majority of the adverse effects of zinc gluconate and of minocycline concerned the gastrointestinal system and weremoderate (5 dropouts with zinc gluconate and 4 with minocycline). Conclusion: Minocycline and zinc gluconate are both effective in the treatment of inflammatory acne, but minocycline has a superior effect evaluated to be 17% in our study.

Background The three-grade acne classification (mild, moderate, severe) is widely used to define the licensed indications of acne treatments, and for therapeutic decision-making in clinical practice, but its reproducibility has never been assessed. Methods Ten photographs of facial acne were scored independently by eight experts using the three-grade acne classification. We conducted a descriptive analysis of the results, based on graphical representation of the scores for each photograph. Results Inter-observer agreement on acne severity based on the three-grade acne classification was very poor. Conclusion The classical three-grade acne classification is poorly reproducible. A new rating tool accompanied by a clinical description of each severity level would be extremely useful.

Existing scoring systems for facial acne focus on the lesions themselves, but clinical decisions are based on a general assessment of severity, including the time since onset, the site(s) of involvement, the patient's history, and the response to prior treatments. The aim of this study was to investigate the influence of some of these factors on the global assessment of acne severity. Involvement of the trunk, prior systemic treatment and a positive family history of acne increased the severity score. Inclusion of these factors could help to compose more homogeneous groups for clinical trials.

Resume Introduction. L'acropathie ulceromutilante de Bureau et Barriere est une complication trophique rare de la neuropathie ethyle, chronique, analgesiante, a l'origine de maux perforants plantaires, de troubles vasomoteurs dysautonomiques et d'arthropathies destructrices. Cette neuroacropathie, appelee « maladie des vagabonds , est le plus souvent associee a un terrain debilite, a un alcoolisme chronique et a des conditions socioeconomiques defavorables. Exegese. Nous rapportons quatre observations qui concernaient des hommes âges en moyenne de 55 ans, dont la presentation se rapprochait des donnees issues de la litterature. En effet, il etaient tous alcooliques, non diabetiques et vivaient dans des conditions d'hygiene precaire. La prise en charge therapeutique efficace des patients a ete difficile en raison d'une mauvaise compliance aux soins et a la poursuite frequente de l'intoxication ethylique. Localement, la mise en decharge du pied est consideree comme la mesure therapeutique principale. Les soins locaux, bains de pieds et pansements aux hydrocolloides doivent etre poursuivis durant plusieurs mois, associes a une antibiotherapie par voie generale dans certains cas. L'evolution est souvent chronique et defavorable. Le retard au diagnostic associe aux possibilites therapeutiques limitees rendent cette affection invalidante, grevee d'une morbidite importante. Conclusion. L'acropathie ulceromutilante est une maladie rare, grave et invalidante. La symptomatologie clinique repose classiquement sur la triade: pied anesthesique, mal perforant plantaire et arthropathies deformantes et mutilantes de l'avant-pied chez des patients souvent alcooliques. La guerison possible est frequemment entravee par une mauvaise compliance aux soins locaux et une poursuite de l'intoxication ethylique.

The objective of this randomized, controlled, multicentre study was to assess the efficacy and safety of a topically applied retinaldehyde 0.1% gel in combination with a topical erythromycin 4% lotion for the treatment of acne vulgaris. Treatment consisted of applying either retinaldehyde or its vehicle every morning and erythromycin every evening for 8 weeks. Efficacy parameters were sequential lesion counts for papules and pustules, and a 6-point semiquantitative scale for comedones and microcysts. Safety parameters were local tolerance and adverse events. Of 74 recruited patients, 73 were appraisable for efficacy and safety. In both treatment groups, papules and pustules were reduced significantly at the end of treatment (P

We report a retrospective study of 14 patients with pityriasis rubra pilaris (PRP) referred to our department from 1973 to 1997. Four of them were children. The purpose of this study was to determine their response to several treatments in order to be able to define the best management of PRP. Nine patients were treated with retinoids (etretinate or acitretin) for an average period of 18.8 months. This treatment achieved in seven of them a partial or complete clearing without major side-effects. All five patients treated with methotrexate were adults. The mean duration of treatment was 12.1 months. All the methotrexate-treated patients improved, even though two of them had not responded to retinoids. The other therapies, including systemic steroids, topical treatments and psoralens-UVA therapy, produced inconsistent results. Our results suggest that retinoids should be considered a first-line treatment at an initial dosage of 0.5 mg/kg per day. Methotrexate has to be the second-line treatment, at an initi...

Several clinical and histopathologic features of 65 CD30+ cutaneous lymphoproliferations were evaluated for their diagnostic value between CD30+ primary versus secondary cutaneous lymphomas and for their prognostic significance. Primary cutaneous disease, spontaneous regression, and absence of extracutaneous spreading (but not age < or =60 years) were associated with a better prognosis. Epithelial membrane antigen, BNH9, CD15 or CBF.78 antigen were expressed in all types of cutaneous lymphoproliferations. However, epithelial membrane antigen immunoreactivity was more frequently expressed in CD30+ secondary cutaneous large-cell lymphoma. Among CD30+ primary cutaneous large-cell lymphoma, CD15 expression was only seen in localized skin lesions. P53 expression was not associated with spontaneous regression, extracutaneous spreading, or survival. Nested reverse transcriptase-polymerase chain reaction allowed the detection of NPM-ALK transcripts in 10 of 26 CD30+ primary and in 3 of 11 secondary cutaneous large-cell lymphomas. The ALK protein was detected in only 1 of 50 primary and in 4 of 15 secondary cutaneous CD30+ lymphoproliferations. In CD30+ primary cutaneous lymphoproliferation, NPM-ALK transcripts might be expressed by very rare normal or tumoral cells that are undetectable by immunohistochemistry. However, the expression of either NPM-ALK transcripts or ALK-protein was not correlated with prognosis or age in CD30+ cutaneous lymphoproliferations.

The sebaceous gland is a target of androgens, and hormonal influences play an important role in the multifactorial pathogenesis of acne. In both men and women, acne may be related to an excessive hypersensitivity of the sebaceous end organ to androgens. However, in women, the ovarian and adrenal production and the plasma transport of androgens may be implicated, particularly in late-onset or persistent acne or with associated hirsutism. Many reports have shown that females with acne frequently have abnormal levels of circulating androgens. Hormonal treatment is not usually the first line in female acne. But in relation to the possibilities, particularly the tetracyclines, this may be a choice when one prescribes a pill like Dianette® to a young woman presenting a mild acne and who requests contraception. However, antiandrogen treatment is sometimes mandatory, especially in resistant acne in adult women and/or in female acne which is resistant or relapsing rapidly after treatment with Roaccutane® and which is often related to hyperandrogenism. Finally, in severe prepubertal acne, the possible cause is an adrenal enzymatic block of which the only treatment is hormonal.

Resume Introduction La forme atrophique de dermatofibrosarcome est une variante rare du dermatofibrosarcome protuberant, ou tumeur de Darier-Ferrand. Elle est preferentiellement observee chez le sujet jeune et l’enfant. Observation Nous rapportons un cas de dermatofibrosarcome multifocal dans sa variante atrophique a debut pediatrique mais diagnostique a l’âge adulte. La tumeur se presentait sous la forme de lesions maculeuses brunâtres de la cuisse droite, totalement asymptomatiques, groupees en une plaque d’environ 10 cm. Devant ce dermatofibrosarcome de grande taille, une chirurgie relativement limitee a ete decidee en premiere intention. Discussion Le diagnostic de dermatofibrosarcome atrophique a debut pediatrique est difficile. Les aspects cliniques sont variables et leur apparente benignite rend souvent le diagnostic tardif. Les examens histologiques et immunohistochimiques aident au diagnostic, et en cas de difficulte, l’etude cytogenetique permet d’apporter un argument pour le diagnostic de dermatofibrosarcome avec la detection du gene de fusion COL1A1- PDGFβ lie a la translocation reciproque t (17, 22). Le probleme est egalement therapeutique, la chirurgie large est le traitement de reference, mais la chirurgie micrographique de Mohs est egalement proposee. Les inhibiteurs du recepteur PDGF tyrosine kinase (imatinib = Glivec ® ) sont reserves aux formes metastatiques.

A multicentre randomized double-blind study of 143 patients was conducted to compare terbinafine (250 mg/day) and micronized griseofulvin (1 g/day) in the treatment of dermatophyte onychomycosis. The duration of treatment was up to 12 months, but the treatment could be interrupted in the event of complete cure, i.e. clinical disappearance of the pathological zone of the nail combined with mycological cure. The percentage of nails cured was significantly higher in the terbinafine than in the griseofulvin group (77.1% versus 45.7%, P=0.0001). Clinical and biological tolerability were good in both groups. Only 7.4% of patients treated with terbinafine showed one or more adverse events, but 31.5% with griseofulvin (P

A 28-year-old man presented with a histiocytic sarcoma of a very uncommon origin, as it had developed for several years like a benign cutaneous histiocytosis resembling generalized eruptive histiocytoma before becoming acute, with nodal and massive pulmonary involvement. Despite various chemotherapies, the patient died within 8 months. Skin biopsies showed histiocytic proliferation in the dermis and node biopsies showed histiocytic proliferation with a sinusoidal pattern. Immunohistochemical analysis, performed on paraffin-embedded sections, demonstrated strong labeling of tumoral cells for CD68 and moderate labeling for CD3 and CD4. CD30 labeling was negative. S-100 protein was positive on a Langerhans' cell reactive subpopulation. Electron microscopy confirmed the histiocytic nature of malignant cells and showed cytoplasmic inclusions such as regularly laminated bodies, dense bodies and pleomorphic inclusions. No Birbeck granules were seen. A gene rearrangement study of T-cell receptor gamma and immunoglobulin heavy chain genes showed a germline configuration. Histiocytic sarcoma is an extremely rare true histiocytic malignancy, the existence of which has been recently debated since it has often been mistaken in the past for large cell lymphomas. Such a deceptive onset as benign cutaneous histiocytosis has not been described in the literature to our knowledge.

Resume Introduction Les proliferations cutanees primitives CD4+ CD56+ constituent une entite rare de description recente, remarquables par leur tropisme cutane primaire et un phenotype CD4+ CD56+ alors que les autres marqueurs B et T sont negatifs. Le nouveau cas rapporte illustre les particularites cliniques et evolutives de cette affection. Observation Un homme de 83 ans avait des lesions papulo-nodulaires infiltrees, ecchymotiques du tronc. La biopsie cutanee d’une lesion montrait une proliferation dermohypodermique de cellules CD3- CD4+ CD56+ CD43+. Il n’y avait pas d’atteinte medullaire, viscerale ou sanguine. Une polychimiotherapie de type CEOP permettait une remission complete apres six cures. Cependant apres quatre mois, les lesions cutanees reapparaissaient, associees a une atteinte sanguine. Apres une reponse initiale a une chimiotherapie de type DHAP, le malade decedait 3 mois plus tard. Discussion L’« agranular CD4+ CD56+ hematodermic neoplasm » est une entite distincte des lymphomes cutanes primitifs. Il s’agit d’une proliferation de cellules derivees de monocytes plasmocytoides predendritiques surexprimant les chaines legeres du CD123. L’histoire naturelle est marquee par un tropisme cutane initial avec des lesions ecchymotiques. Les polychimiotherapies sont inefficaces avec un echappement constant. Un espoir pourrait consister en l’utilisation d’un anticorps antiCD123 specifique des monocytes plasmocytoides.

Sweet's syndrome is an acute febrile neutrophilic dermatosis. Although it frequently appears as an idiopathic disorder, it may occur in association, often as presenting sign, with malignancy or more rarely with infections. We report two cases of Sweet's syndrome preceded by digestive infection due to Yersinia enterocolitica, affirmed by significant rises in serum antibody titers. Other nongastrointestinal manifestations of such infections are known, predominantly arthritis and erythema nodosum. Sweet's syndrome is a rare complication of these infections. Treatment with systemic steroids, usually effective, can be replaced by antibiotics with apparently favorable results. The search of an infectious origin should be systematic in cases of Sweet's syndrome that appear to be idiopathic.

The hepatitis C virus causes both hepatic and extrahepatic disorders, particularly as regards dermatology. The link between essential mixed cryoglobulinemias and the C virus infection has been clearly evidenced., whereas its frequency seems low in other systemic vasculitis such as polyarteritis nodosa. Similarly, the link between C virus hepatopathy and porphyria cutanea tarda is now proven. Lichen planus is also described as being associated with this virus, but further epidemiological studies are required to determine the exact prevalence of lichen in C virus hepatopathy cases. Finally, various cutaneous disorders, such as urticaria, erythema multiforme, dermo-hypodermitis, etc, occasionally arise during acute or chronic hepatitis C.

Le tissu conjonctif est largement present dans l’organisme. Ce tissu de soutien proteiforme possede toutefois une certaine specificite d’organe dans son organisation structurale et sa composition moleculaire. Ainsi, dans la peau, le derme comprend une matrice extracellulaire associant principalement des fibres de collagene et des fibres elastiques baignant dans la substance fondamentale. Les fibres de collagene cutanees sont principalement composees des collagenes I, III et V. Le type I y est le plus abondant. Le type III est majoritaire dans le derme papillaire; il est egalement present dans les parois vasculaires et dans celles des organes digestifs. La composition en acides amines et la structure en triple helice font des collagenes des modeles uniques de proteines fibreuses. Les fibres collagenes dermiques assurent la resistancemecanique de la peau et n’ont pas d’orientation precise. Les fibres elastiques sont responsables de l’elasticite cutanee.Les fibres pre-elastiques du derme papillaire, oxytalanes et elaunines, sont essentiellement composees de glycoproteinesmicrofibrillaires, en particulier les fibrillines 1 et 2.

Background: Recent advances in the treatment of psoriasis include both the topical vitamin D analogue calcipotriol and cyclosporine. Combined treatments have been sought to decrease the incidence of side effects while maintaining efficacy in the treatment of severe chronic plaque psoriasis. Objective: The objective of this study was to evaluate the efficacy and safety of the combination of 2 mg/kg/day of cyclosporine with calcipotriol ointment (50 μ g/gm) in the treatment of severe plaque psoriasis. Methods: Sixty-nine patients were randomly selected for this double-blind, multicenter study to receive cyclosporine (2 mg/kg/day) combined with calcipotriol ointment (50 μ g/gm) or cyclosporine (2 mg/kg/day) combined with placebo ointment (vehicle of calcipotriol) for a 6-week period. Results: Complete clearing or 90% improvement in Psoriasis Area and Severity Index score occurred in 50.0% of patients in the calcipotriol/cyclosporine group in comparison with 11.8% of patients treated with placebo/cyclosporine ( p = 0.0019). The confidence interval for the difference ranged from 17.8% to 58.7%. No difference was found between the two groups with respect to side effects. Conclusion: The calcipotriol/cyclosporine combination was more effective than placebo/cyclosporine. Further studies are needed to establish the long-term efficacy and safety profile of this combination therapy.

Arterial involvement is an important feature of the diagnosis and, above all, prognosis of heritable disorders of connective tissue. In pseudoxanthoma elasticum, a progressive occlusive syndrome is associated with hemorrhage and especially with gastrointestinal bleeding. Aneurysms are uncommon. Hypertension occurs frequently. Cutaneous signs (yellowish pseudo xanthomatous papules of the large folds) the ocular changes (angioid streaks) and pathology showing numerous, thickened, fragmented, disorganized, calcified elastic fibers in the deep dermis and arterial walls, allow the diagnosis to be made. In the heterogeneous group of Ehlers-Danlos syndromes, type IV is characterized by sudden spontaneous rupture of the large arteries. Aneurysms and carotido-cavernous fistulae are rather frequent. Owing to friability of the arterial walls, arteriograms and other procedure requiring arterial puncture may prove hazardous and surgery difficult. Such patients have an acrogeric morphotype, and thin, fragile skin, but cutaneous hyperelasticity and joint hyperlaxity are usually minimal. Pathology evidences collagen hypoplasia in the skin and arterial walls. The severity of Marfan syndrome is due to aortic involvement. A fusiform aneurysm of the ascending aorta represents a vital risk of rupture. Aortic root dilatation is associated and responsible of severe aortic regurgitation. Aortic dissection is also a serious threat. Improved surgical techniques for repairing a dilated or dissected aortic root with simultaneous replacement of the aortic valve increases the life expectancy of such patients. Dolichomorphism is the characteristic skeletal abnormality, particularly with arachnodactyly and upward ectopia lentis, which is almost bilateral, is a very frequent feature of Marfan syndrome. The most typical histological finding is aortic cystic median necrosis. The basic defect in Marfan syndrome concerns the fibrillin, whose gene is located on chromosome 15. The three diseases detailed in this paper constitute the main areas of this subject, but arterial involvement may occur in other inheritable disorders of connective tissue (osteogenesis imperfecta, cutis laxa, Werner syndrome, Menkes syndrome, etc).

Resume La bronchiolite obliterante est exceptionnelle au cours du lupus. Il n'existe pas de schema therapeutique et son pronostic reste sombre. Nous rapportons le cas d'une jeune fille de 18 ans, atteinte d'une bronchiolite obliterante (pas d'histologie specifique) pour laquelle plusieurs traitements ont ete tentes sans succes : corticotherapie orale, plasmaphereses, bolus de cyclophsophamide. Seuls les bolus de methylprednisolone ont permis une nette amelioration de son etat respiratoire.

Acne can have an important psychological impact. We surveyed 852 adolescents aged 12-25 years about their knowledge of acne and its treatment in a non-medical context. The study involved a questionnaire administered to callers to a youth telephone helpline in France. Callers were categorized into those who currently had acne, those who had had acne previously, and those who had never had acne. Most respondents (66.2%) had experienced acne symptoms, which were mild in 50.2% of cases and severe in 16% of cases. Often, acne had been long-lasting (>12 months in 49.6% of cases). Many thought that gender, excess weight, eating dairy products, and physical activity did not influence acne, and that frequent washing could improve acne. Eating chocolate and snacks, smoking cigarettes, sweating, not washing, touching/squeezing spots, eating fatty foods, using make-up, pollution, and menstruation were thought to worsen acne. The majority (80.8%) did not believe acne to be a disease, but rather a normal phase of adolescence, yet 69.3% agreed it should be treated. There was a preference for topical vs. systemic treatment. Many (38.6%) of the respondents with acne had not consulted a physician. Almost two-thirds of respondents wanted more information about acne. Providing more information about acne might increase the likelihood of them consulting a physician and getting better treatment for the condition.

Background: The biologically active form of vitamin D3, calcitriol, may offer a new therapeutic approach to psoriasis, Calcipotriol, a new vitamin D3 analogue, is at least 100 times less calcemic than calcitriol. Objective: Our purpose was to study the efficacy and safety of calcipotriol in the treatment of psoriasis vulgaris. Methods: In a right/left comparative, double-blind study, treatment with calcipotriol ointment (50 μg/gm) twice daily and placebo was given for 4 weeks. The preferred treatment was continued, without opening the code, for another 4 weeks. Efficacy, as measured by the Psoriasis Area and Severity Index and by the investigator's and patient's global assessment, and safety were assessed every 2 weeks. Results: The mean Psoriasis Area and Severity Index fell in 4 weeks from 14.2 to 6.3 with calcipotriol and from 14.1 to 9.2 with placebo (p Conclusion: Topical application of up to 50 gm of calcipotriol ointment per week was found to be an effective and safe treatment of psoriasis vulgaris.

Resume Les auteurs rapportent 2 cas de paralysie faciale peripherique chez des sujets presentant une infection a VIH. A propos de ces 2 observations et de celles de la litterature, sont rappeles les caracteres cliniques et paracliniques de cette atteinte du VII. Les differentes hypotheses etiologiques sont discutees mais il est probable qu'il s'agisse en fait d'une atteinte directe du nerf par le virus. Ces paralysies faciales peuvent survenir quelque soit le chiffre des lymphocytes CD4 et ne semblent pas avoir de signification pronostique. Elles apparaissent cependant preferentiellement aux stades I, II et III, pouvant alors etre le mode de revelation de l'infection par le VIH.

Leprosy is now considered in Western Europe as a strictly imported disease. A case of presumed locally acquired diffuse lepromatous leprosy was observed in a native-Portuguese woman living in France who developed an acute febrile presentation with extensive cutaneous signs.

We report the cases of 4 male subjects, 29, 32, 41 and 44 years old, presenting isolated seropositivities for the human immunodeficiency virus (HIV), or full-blown acquired immunodeficiency syndrome, associated with a typical porphyria cutanea tarda (PCT). The 4 patients are in the usual risk groups for HIV infection. Viral hepatitis was observed in 3 of the 4 cases. Over the past 3 years, 15 cases associating HIV infection and PCT have been reported; almost all had the usual risk factors for HIV infection and hepatopathy. We speculate that HIV infection may have favored the occurrence of early PCT in these cases by altering the metabolism of the porphyrins, either directly or by means of the associated hepatopathy.

We present a case of dermatophytic granuloma caused by Microsporum canis in a heart-lung recipient. This 66-year-old man was seen for erythematous pustules and papules on the forearm. The diagnosis was suspected after histological examination showing an inflammatory infiltrate in the upper dermis with giant cells containing intracytoplasmic fungal elements. Cultures of the skin biopsy confirmed the diagnosis identifying M. canis. Our case emphasizes the possibility of deep dermatophytic infections in immunocompromised patients. There are only 4 additional reports of M. canis infection responsible for invasion of the dermis in such patients. The follicle involvement probably explains these dermal lesions due to the progression of the dermatophyte from the hair follicle to the dermis. In our observation topical antifungal therapy alone was unsuccessful and fluconazole seems to be the treatment of choice for these M. canis invasive dermal cutaneous infections.

We report a case of subcorneal pustular dermatosis (SPD) first treated with dapsone with poor response. Dapsone was changed to acitretin, which dramatically improved the lesions in a few days. Dapsone is the first-line treatment in SPD, but it may be ineffective in some cases and its toxicity is important. Therapeutic alternatives are limited and less effective (systemic corticosteroids, phototherapy). Our observation underlines the usefulness of retinoids, when dapsone is ineffective or poorly tolerated. Their effectiveness is comparable, but they are effective more rapidly and are better tolerated. A dose maintenance is necessary to avoid relapses. Their action in SPD remains unclear but may be due to the inhibition of neutrophil functions.

Practicing dermatologists rarely evaluate therapeutic trials. This study was designed to build a simple evaluation tool, use it to evaluate the quality of published trials and thereby consider their applicability. Reports on 146 therapeutic trials, published from 1969 to 2004, were selected. A 50-item list was used for an initial assessment. After comparison with existing grids, a shortened nine-item list was established and used for this analysis. The results were analyzed item by item (logistic regression) and globally (principal component analysis). An index was then established and validated using Cronbach's coefficient alpha. The overall quality of trials was poor: 9% to 73% positive response range. This rate increased after 1990 for six items. Two factors explained almost 60% of the total variance: factor 1 summing six items concerning the formal content of the articles and factor 2 its underlying basis. Using the sum of these six variables, Cronbach's coefficient alpha reached 0.716; their mean sum improved from 1969 to 1970-2001-2005. The significantly improved quality of trials, based on the mean sum over time, supports well-founded basis of this tool. Regarding the application of evidence-based medicine in clinical practice, this tool is user friendly and should facilitate the updating of our knowledge.

Acne is a disorder in which adherence has a major impact on treatment outcome.To create an easy-to-use tool to assess treatment adherence, based on a questionnaire that can be used on a daily basis by dermatologists.First, we evaluated adherence by comparing responses to a self-administered questionnaire with the dermatologist's prescription. Second, concordance was assessed between adherence and each question of a dermatologist-directed questionnaire (DDQ) administered during the consultation. Third, DDQ questions were combined to construct a mini-questionnaire, and these combinations were tested to maximize specificity.The study included 246 patients, who were prescribed topical treatment alone, combined treatment (excluding oral isotretinoin) or oral isotretinoin alone. The mini-questionnaires for oral and topical treatments had 89% specificity for detecting poor adherence. An inappropriate answer to one question classified the patient as a probable poor compliance.Our mini-questionnaires provide dermatologists with specific tools to rapidly identify poor compliance.

Acne can have important psychological repercussions.We studied adolescents' perceptions of acne, in a nonmedical context.Between November 2004 and January 2005, a questionnaire was presented to adolescents calling a youth helpline. It focused on perceptions of acne and, among callers with personal experience of acne, its severity and treatment. Callers were divided into those with acne, those having had acne and those never having had acne.The questionnaire was completed by 1,566 callers. It showed that 51% of respondents with acne did not seek medical help; acne has as big a psychological impact as some far severer diseases; many respondents with no history of acne said they were very worried that they would develop this skin disorder.This study underlines the major psychological impact of acne. Young people need to be informed that acne can be treated.