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Resume Introduction Nous rapportons un cas de DRESS original par sa presentation clinique. Observation Un homme de 52 ans traite par methotrexate et celecoxib pour un rhumatisme inflammatoire et depuis 2 jours par amoxicilline-acide clavulanique etait hospitalise pour une eruption febrile pustuleuse couvrant 80 % de la surface corporelle, avec des placards infiltres couverts de pustules. Dans les jours suivants, la fievre persistait, associee a une polyadenopathie, une cytolyse hepatique, une hypereosinophilie, une pneumopathie interstitielle et une atteinte cardiaque. La biopsie cutanee etait compatible avec une toxidermie. La PCR-EBV etait positive. Le diagnostic de DRESS etait retenu avec un score REGISCAR > 5. Une corticotherapie generale etait debutee et permettait l’apyrexie en 48 h et la regression de l’eruption, des anomalies biologiques et une recuperation de la fonction cardiaque. Les traitements etaient contre-indiques jusqu’a realisation des tests allergologiques. Discussion Le cas est original par sa presentation pustuleuse, mimant une pustulose exanthematique aigue generalisee initiale et une atteinte cardiaque avec troubles de la conduction et alteration de la fonction ventriculaire. La presence du virus EBV pose ici la question de son implication causale dans le tableau clinique. Aucun cas de DRESS au methotrexate n’est decrit. La chronologie ne permet pas d’imputer l’amoxicilline mais on ne peut eliminer son role en tant que cofacteur dans l’exacerbation des symptomes et la reactivation virale a EBV. Conclusion Nous rapportons un cas de DRESS original par sa presentation cutanee pustuleuse et les signes cardiaques associes. L’implication causale du virus EBV est suspectee en association probable avec l’amoxicilline.

El carcinoma de Merkel es un tumor maligno neuroendocrino cutaneo primitivo que pertenece a los tumores cutaneos raros. El tumor afecta de manera mayoritaria a los varones mayores de 65 anos. La edad, la exposicion cronica a los ultravioletas y la inmunodepresion son factores de riesgo conocidos. La presentacion clinica a menudo es monomorfa, con un tumor nodular, eritematoso, firme y subcutaneo, aunque se describen formas atipicas. La anatomia patologica muestra un tumor dermico compuesto por celulas pequenas basofilas, con numerosas mitosis. El diagnostico histologico se basa en la inmunohistoquimica, que es positiva para citoqueratina 20 y negativa para citoqueratina 7 y factor de transcripcion tiroideo 1 (TTF1).Un poliomavirus, el poliomavirus de celulas de Merkel, esta implicado en la oncogenesis del tumor de manera concluyente, sin impacto sobre el tratamiento actualmente. Existe una asociacion demostrada entre el carcinoma de Merkel y otros canceres, en particular cutaneos, y las hemopatias. El estudio inicial durante el diagnostico de carcinoma de Merkel debe comprender una exploracion fisica completa, en particular cutanea, en busca de otros canceres cutaneos, asociado a una ecografia de las areas ganglionares de la zona de drenaje del tumor y a una tomografia computarizada toracoabdominopelvica. La busqueda del ganglio centinela se ha convertido en estandar. El riesgo de recidiva del tumor se estima en un 30-50% localmente, un 40% en los ganglios y un 10-35% a distancia. El tratamiento del tumor a escala localizada se basa en la exeresis quirurgica en margenes sanos asociada a una radioterapia local. Puede discutirse la radioterapia profilactica ganglionar. Si el ganglio centinela esta invadido, se propone un vaciamiento ganglionar. En el estadio ganglionar, el tratamiento asocia cirugia a vaciamiento ganglionar y radioterapia. En el estadio metastasico, no existe ninguna recomendacion. La quimioterapia convencional ha demostrado un porcentaje elevado de respuestas, pero con recaida rapida. Recientemente, la inmunoterapia ha demostrado su eficacia.

CARADERM is a French national network that includes patients with rare skin adnexal neoplasms. The present paper describes only the adnexal neoplasm part of this network. The primary objective of CARADERM is to improve medical care for malignant skin adnexal neoplasms. A multidisciplinary review group and a centralized pathological review group have been set up.A dual network of clinicians and pathologists has been set up. Data are recorded in a secure database.The CARADERM network comprises of 38 clinical centres and 22 pathology centres. Between 2014 and 2017, 1598 patients with an adnexal neoplasm were included. Data of interest were documented in 80% of cases. Median patient age was 72 years. Major histological subtypes were sweat gland carcinomas (50%), hair follicle carcinomas (37.7%), and sebaceous gland carcinomas (9.8%). Surgery was the first-line treatment for 81% of patients, including 76.9% with standard surgical margin analysis, and 5.5% with exhaustive margin analysis. 920 patients (57.6%) underwent a national pathology review process.The CARADERM network aims at providing assistance in difficult situations concerning diagnosis and care in skin adnexal neoplasms. Analysis of the CARADERM data should allow the creation of a prognostic classification of these rare neoplasms together with recommendations. A national multidisciplinary consensus exists. Translational and therapeutic research is ongoing.The CARADERM network is currently recruiting and more data should lead to improved knowledge of these tumours in the coming years.

Improved knowledge of sentinel node procedures coupled with the results of adjuvant clinical trials in stage III melanoma have prompted the French Cutaneous Oncology Group to propose new guidelines for the management of stage III melanoma. These guidelines comply with the principles of the evidence-based medicine.

The recent publication of randomized trials investigating the efficacy of adjuvant therapy and completion lymph node dissection at microscopic stage III melanoma calls for a reappraisal of melanoma management from different angles: indications for sentinel lymph node biopsy, indications for completion lymph node dissection in microscopic-stage disease, and adjuvant therapies. Our objective was to evaluate current practices and to question French onco-dermatologists about any changes they envisaged in their practices in the light of recent publications.We conducted a national survey among members of the Cutaneous Oncology Group of the French Society of Dermatology in October 2017.Forty French health centers were included, and 53 individual responses were collected. Sentinel lymph node biopsy for melanoma was performed at 75 % of the centers. Before the summer of 2017 and the publication of MSLT-II (proving the absence of any therapeutic benefits for complete lymph node dissection in microscopic stage III melanoma), when a positive sentinel lymph node was diagnosed, immediate completion lymph node dissection was performed at 90 % of the centers. After the publication of MSLT-II, 45 % of the respondents considered stopping this practice. The risk-benefit ratio prompted prescription of nivolumab and of combined dabrafenib+trametinib as adjuvant therapy by respectively 96 % and 79 % of respondents, while the corresponding rates for interferon and ipilimumab were only 21 % and 15 %.Early melanoma management stands on the verge of major changes thanks to the arrival of efficient adjuvant therapies and a decrease in immediate completion lymph node dissections for patients with microscopic stage III is also anticipated.

An original case of DRESS syndrome is reported herein with a particular cutaneous presentation and etiology.A 52-year-old man developed a febrile pustular rash after being treated with methotrexate and celecoxib for inflammatory rheumatism and with amoxicillin-clavulanic acid over the previous 2 days. Eighty percent of his body surface was covered with pustular infiltrated plaques. On the following days, the patient developed persistent fever, with polyadenopathy, hepatic cytolysis, eosinophilia, interstitial lung disease and cardiac involvement. Cutaneous biopsy was consistent with a drug eruption. Epstein Barr Virus PCR was positive. A diagnosis of DRESS syndrome was made with a RegiSCAR score above 5. Systemic corticosteroids were given, resulting in cessation of the fever and complete recovery with regard to dermatosis, laboratory abnormalities and cardiac function.The present case is original, with a febrile pustular eruption mimicking acute generalized exanthematous pustulosis and cardiac anomalies with electrical changes and impairment of ventricular function. Epstein Barr Virus may have played a role in the presentation. No previous cases of DRESS syndrome caused by methotrexate have been described. Amoxicillin was probably not involved in the present case of DRESS syndrome as it was taken for only two days, but its role as a co-factor in association with the EBV viral reactivation should not be ruled out.We report an original case of DRESS syndrome in terms of pustular cutaneous presentation and cardiac impairment. EBV reactivation associated with amoxicillin may be suspected.

Resume Introduction Au cours de cette etude retrospective, nous voulions evaluer la faisabilite de la technique du ganglion sentinelle au cours de la prise en charge des carcinomes de Merkel de la face. Materiel et methodes Notre serie comportait 12 patients pris en charge pour un carcinome de Merkel de la face et qui ont beneficie de la technique du ganglion sentinelle sur 4 ans. Resultats Parmi les 12 patients inclus, 1 seul presentait un ganglion sentinelle positif. Le ganglion sentinelle n’a pas ete identifie chez 3 patients. Sur les 8 patients restants, 2 patients qui presentaient un ganglion sentinelle negatif ont presente une recidive ganglionnaire cervicale dans les 2 ans. Une patiente est decedee durant le suivi. Conclusion Le carcinome de Merkel est une pathologie rare mais agressive et les protocoles de prise en charge sont bases sur de petites series. Le role du ganglion sentinelle au cours de la prise en charge du ganglion sentinelle reste a definir.

Introduction A retrospective preliminary study assessed the feasibility of sentinel lymph node biopsy (SLNB) in the management of Merkel cell carcinoma (MCC) of the head and neck. Patients and methods Twelve patients with stage I or II head and neck MCC underwent SLNB over a 4-year period. Results Only 1 of the 12 patients had a positive SLNB. The sentinel node was not identified in 3 patients. Two of the 8 patients with negative SLNB showed regional lymph node recurrence within 2 years. One patient died during follow-up. Conclusion Merkel cell carcinoma is an uncommon but highly aggressive pathology; management protocols have been based on small series. The role of SLNB in the management of MCC remains to be defined.

Resume Introduction Le pemetrexed (Alimta®) est un nouvel agent anti-tumoral de la classe des anti-folates indique dans le traitement du mesotheliome pleural malin et du cancer bronchique non a petites cellules (CBNPC). Nous rapportons deux nouveaux cas de toxicite cutanee induite par le pemetrexed, a type de sclerose cutanee des jambes. Observations Cas no 1 : un homme de 66 ans presentait un adenocarcinome bronchique d�emblee metastatique, traite par six cures de cisplatine-pemetrexed, puis par pemetrexed seul en traitement d�entretien. Apres le quatrieme cycle de pemetrexed, il presentait une dermo-hypodermite douloureuse des membres inferieurs, non febrile, ressemblant cliniquement a un erysipele. Cas no 2 : une femme de 70 ans recevant du pemetrexed en traitement d�entretien pour un CBNPC presentait des le premier cycle un �deme erythemateux des jambes. Il s�agissait de placards erythemato-violaces �demateux, indures et douloureux, associes a une impotence fonctionnelle. Dans les deux cas, le pemetrexed a ete arrete et l�evolution locale a ete lentement favorable, avec persistance d�un �deme sclereux. Discussion Cet effet indesirable cutane est meconnu, entrainant un retard diagnostique. Les lesions sont initialement confondues avec un erysipele malgre la bilateralite et l�absence de syndrome febrile. Une supplementation par folates et vitamine B12 associee a de la dexamethasone pourrait faire diminuer l�incidence et la severite de cette toxicite. Nos deux patients n�ont pas presente de recidive ni d�aggravation de leur scler�deme sous taxanes, agents de chimiotherapie connus pour etre pourvoyeur de syndromes sclerodermiformes. Nous estimons que cette toxicite cutanee doit etre connue en raison de sa severite potentielle. Summary Background Pemetrexed (Alimta®) is a new-generation antifolate used to treat malignant pleural mesothelioma and non-small cell lung cancer (NSCLC). We report two cases of a new toxicity induced by pemetrexed: scleroderma-like induration of the lower extremities. Patients and methods The first case concerned a 66-year-old man diagnosed with pulmonary adenocarcinoma metastatic from the outset and in whom maintenance treatment comprised pemetrexed after first-line therapy comprising six courses of cisplatin-pemetrexed. After the fourth cycle of pemetrexed, he presented an erythematous oedema of the left leg, which was subsequently bilateral. Clinically, there was painful cellulitis associated with areas of bruising. The lesions had an appearance of erysipeloid-like infection, and there was no fever. The second case concerned a 70-year-old woman diagnosed with metastatic NSCLC. From the first course of pemetrexed, given as maintenance therapy, she presented erythematous oedema of both legs, without fever. After the second course, we observed the recurrence of the lesions consisting of erythemato-violaceous plaques on both legs, with severe bilateral indurated and painful oedema, associated with major functional disability. A diagnosis of bilateral erysipelas was made, and antibiotic treatment with cloxacillin was given. In both cases, pemetrexed was discontinued and the local outcome was very slowly favourable, with persistence of scleroderma. Discussion This cutaneous adverse effect is unrecognized, resulting in delayed diagnosis. It is often initially confused with bilateral erysipelas, despite absence of fever. According to some studies, the severity of the cutaneous toxicity may be connected with patients� folate status. Thus folate and vitamin B12 supplementation combined with dexamethasone could decrease the incidence of this side effect. There was no recurrence and no worsening with taxanes, chemotherapy agents known to induce scleroderma. We feel that this cutaneous toxicity must be recognised on account of its potential severity.

Introduction Les immunotherapies sont souvent responsables d’effets indesirables auto-immuns parmi lesquels les dysthyroidies. Methode Nous avons mene une etude retrospective de la cohorte de patients traites par immunotherapie entre aout 2014 et fevrier 2017 sur le centre hospitalier de Pau afin d’evaluer la prevalence et le mecanisme de ces dysthyroidies. Resultats Quatre-vingt-deux patients ont recu un traitement par immunotherapies dont 61 du nivolumab, 14 de l’ipilimumab et 13 du pembrolizumab (indication : melanome ou cancer pulmonaire non a petites cellules metastatique). Dix-sept pour cent ont presente une dysthyroidie. Une thyrotoxicose etait observee chez 10 patients (11,4 %) dont 9 sous nivolumab et 1 sous ipilimumab. L’hyperthyroidie etait precoce (3e cure en mediane) fruste dans 70 % des cas et transitoire (1,5 mois). Deux patients ont beneficie d’un traitement specifique. Seule la patiente sous ipilimumab presentait des TRAK et des ATPO. Trois patients ont eu une scintigraphie : deux retrouvees normales, une blanche. Trois patients ont evolue vers l’hypothyroidie. Trois patients supplementaires ont presente une hypothyroidie sans thyrotoxicose prealable (2 sous ipilimumab, 1 sous pembrolizumab). Le delais median de survenue etait la 5e cure. Il n’a pas ete retrouve chez ces patients d’anticorps antithyroidiens. Presenter une dysthyroidie etait associe a une amelioration non significative de la survie globale. Conclusion Notre etude retrouve une forte prevalence des effets indesirables thyroidiens des immunotherapies. Seule une patiente presentait des anticorps specifiques, ce qui est contraire aux donnees de la litterature. Cela peut-etre en lien avec un dosage des anticorps parfois realise plusieurs semaines apres apparition des premieres anomalies biologiques.

Pemetrexed (Alimta(®)) is a new-generation antifolate used to treat malignant pleural mesothelioma and non-small cell lung cancer (NSCLC). We report two cases of a new toxicity induced by pemetrexed: scleroderma-like induration of the lower extremities.The first case concerned a 66-year-old man diagnosed with pulmonary adenocarcinoma metastatic from the outset and in whom maintenance treatment comprised pemetrexed after first-line therapy comprising six courses of cisplatin-pemetrexed. After the fourth cycle of pemetrexed, he presented an erythematous oedema of the left leg, which was subsequently bilateral. Clinically, there was painful cellulitis associated with areas of bruising. The lesions had an appearance of erysipeloid-like infection, and there was no fever. The second case concerned a 70-year-old woman diagnosed with metastatic NSCLC. From the first course of pemetrexed, given as maintenance therapy, she presented erythematous oedema of both legs, without fever. After the second course, we observed the recurrence of the lesions consisting of erythemato-violaceous plaques on both legs, with severe bilateral indurated and painful oedema, associated with major functional disability. A diagnosis of bilateral erysipelas was made, and antibiotic treatment with cloxacillin was given. In both cases, pemetrexed was discontinued and the local outcome was very slowly favourable, with persistence of scleroderma.This cutaneous adverse effect is unrecognized, resulting in delayed diagnosis. It is often initially confused with bilateral erysipelas, despite absence of fever. According to some studies, the severity of the cutaneous toxicity may be connected with patients' folate status. Thus folate and vitamin B12 supplementation combined with dexamethasone could decrease the incidence of this side effect. There was no recurrence and no worsening with taxanes, chemotherapy agents known to induce scleroderma. We feel that this cutaneous toxicity must be recognised on account of its potential severity.

BACKGROUND Combined BRAF and MEK inhibition, as compared with BRAF inhibition alone, delays the emergence of resistance and reduces toxic effects in patients who have melanoma with BRAF V600E or V600K mutations. METHODS In this phase 3 trial, we randomly assigned 423 previously untreated patients who had unresectable stage IIIC or stage IV melanoma with a BRAF V600E or V600K mutation to receive a combination of dabrafenib (150 mg orally twice daily) and trametinib (2 mg orally once daily) or dabrafenib and placebo. The primary end point was progression-free survival. Secondary end points included overall survival, response rate, response duration, and safety. A preplanned interim overall survival analysis was conducted. RESULTS The median progression-free survival was 9.3 months in the dabrafenib–trametinib group and 8.8 months in the dabrafenib-only group (hazard ratio for progression or death in the dabrafenib–trametinib group, 0.75; 95% confidence interval [CI], 0.57 to 0.99; P = 0.03). The overall response rate was 67% in the dabrafenib–trametinib group and 51% in the dabrafenib-only group (P = 0.002). At 6 months, the interim overall survival rate was 93% with dabrafenib–trametinib and 85% with dabrafenib alone (hazard ratio for death, 0.63; 95% CI, 0.42 to 0.94; P = 0.02). However, a specified efficacy-stopping boundary (two-sided P = 0.00028) was not crossed. Rates of adverse events were similar in the two groups, although more dose modifications occurred in the dabrafenib–trametinib group. The rate of cutaneous squamous-cell carcinoma was lower in the dabrafenib–trametinib group than in the dabrafenib-only group (2% vs. 9%), whereas pyrexia occurred in more patients (51% vs. 28%) and was more often severe (grade 3, 6% vs. 2%) in the dabrafenib–trametinib group. CONCLUSIONS A combination of dabrafenib and trametinib, as compared with dabrafenib alone, improved the rate of progression-free survival in previously untreated patients who had metastatic melanoma with BRAF V600E or V600K mutations. (Funded by GlaxoSmithKline; Clinical Trials.gov number, NCT01584648.)

Vitiligo/nonsegmental vitiligo (NSV) is often associated with thyroid dysimmunity although very few reports have studied this association using multivariate logistic regression.To identify weighted factors associated with the presence of autoimmune thyroid disease (AITD) in a large cohort of patients with vitiligo/NSV.This was a prospective observational study in 626 patients with a confirmed diagnosis of vitiligo/NSV attending the vitiligo clinic of the University Hospital Department of Dermatology, Bordeaux, France, from 1 January 2006 to 1 May 2012. The Vitiligo European Task Force (VETF) questionnaire was completed for each consecutive patient. AITD was defined as the presence of significant levels of serum antithyroperoxidase antibodies or evidence of autoimmune thyroiditis. Univariate and multivariate logistic regression procedures were conducted to identify factors associated with AITD in this cohort of patients with vitiligo/NSV.A total of 626 patients with vitiligo/NSV were included, of whom 131 had AITD (AITD-vitiligo). Stress as an onset factor, familial history of AITD, body surface involvement and duration of the disease were positively associated with AITD-vitiligo using univariate analysis, whereas female sex, age at onset of vitiligo, personal history of autoimmune disease and localization on the trunk were found to be independently associated with AITD-vitiligo.Vitiligo associated with AITD has clinical features distinct from vitiligo without AITD. In particular, female patients, and patients with longer duration of disease and greater body surface involvement are more likely to present with AITD and should thus be monitored for thyroid function and antithyroid antibodies on a regular basis.

The aetiopathogenic mechanisms of vitiligo are still poorly understood, and this has held back progress in diagnosis and treatment. Up until now, treatment guidelines have existed at national levels, but no common European viewpoint has emerged. This guideline for the treatment of segmental and nonsegmental vitiligo has been developed by the members of the Vitiligo European Task Force and other colleagues. It summarizes evidence-based and expert-based recommendations (S1 level).

Limited epidemiological data exist that compare clinical features of pre- and post-pubertal nonsegmental vitiligo.To compare factors associated with pre- and post-pubertal onset vitiligo.A prospective observational study was conducted of patients with vitiligo attending the clinic between 1 January 2006 and 1 July 2011. The Vitiligo European Task Force questionnaire was completed for each patient and thyroid function and antithyroid antibodies were screened. Other forms of vitiligo (segmental, focal, mucosal, not classifiable) were excluded.A total of 679 patients were included; 422 had post-pubertal and 257 pre-pubertal onset of vitiligo. Vitiligo universalis was seen only in post-pubertal onset. In univariate analysis, there was no significant statistical difference for sex, Koebner phenomenon or disease activity between both groups; thyroid disease or presence of thyroid antibodies was more frequent in post-pubertal onset [odds ratio (OR) 0·31, P0·003] whereas atopic dermatitis was more often associated with or preceding pre-pubertal onset (OR 2·42, P = 0·006). In multivariate analysis, halo naevi, family history of vitiligo, premature hair greying, atopic dermatitis and previous episode of spontaneous repigmentation were independently associated with pre-pubertal onset. In contrast, stress as onset factor, personal history of thyroid disease and acrofacial type were associated with post-pubertal onset.Pre-pubertal onset vitiligo is strongly associated with personal and family history of atopy, suggesting that the predisposing immune background in vitiligo is not limited to autoimmunity, as also noted in alopecia areata. This study also suggests reconsidering the epidemiological data on sex ratio in vitiligo.

Until now, segmental vitiligo has been considered as a stable entity and mixed vitiligo, the association of segmental and nonsegmental vitiligo, has been reported rarely.The aim of this study was to search for factors associated with the generalization of vitiligo in patients with segmental vitiligo.This was a prospective observational study conducted in the vitiligo clinic of the Department of Dermatology of Bordeaux, France. The Vitiligo European Task Force questionnaire was completed for each patient attending the clinic with a confirmed diagnosis of segmental vitiligo after exclusion of other forms of vitiligo (focal, mucosal, not classifiable.) Thyroid function and antithyroid antibodies were screened if not obtained in the previous year.One hundred and twenty-seven patients were recruited: 101 had segmental vitiligo and 26 had segmental vitiligo that evolved into mixed vitiligo; 56 were male and 71 were female. Most patients had onset of segmental vitiligo before the age of 18. When conducting multivariate analysis, we found the following to be independent factors associated with the evolution of patients' disease from segmental vitiligo to mixed vitiligo: initial percentage of body surface involvement of the segment1% [odds ratio (OR) 15·14, P=0·002], the presence of halo naevi (OR 24·82, P=0·0001) and leukotrichia (OR 25·73, P=0·0009).Halo naevi association and leukotrichia at first consultation in segmental vitiligo are risk factors for the progression of segmental vitiligo to mixed vitiligo. In addition, this progression of segmental vitiligo to mixed vitiligo carries a stronger link if initial segmental involvement is situated on the trunk.

Although mixed forms have been described recently, segmental (SV) and nonsegmental vitiligo (NSV) are considered as clinically distinct. However, limited epidemiological data are available to help distinguish associated factors, and recent genome-wide association studies have been restricted to NSV. The higher prevalence of SV in children is helpful when comparing the two major presentations of the disease.To compare factors associated with SV and NSV, especially for markers of autoimmunity or autoinflammation.We conducted a single-centre prospective observational study in patients aged 17 years or under with a confirmed diagnosis of SV or NSV at the vitiligo clinic between 1 January 2006 and 1 July 2010. The Vitiligo European Task Force questionnaire was completed for each patient, and thyroid function and antithyroid antibodies were screened if not obtained in the previous year. Other forms of vitiligo (focal, mucosal, not classifiable) were excluded.A total of 213 children were included, 142 with NSV, 59 with SV and 12 with mixed vitiligo. There was no significant statistical difference for sex or age at onset between patients with SV and NSV. Halo naevi were significantly more frequent in NSV than in SV [odds ratio (OR) 7·58, P0·01). Patients with NSV more frequently had a positive family history of vitiligo (OR 2·25, P=0·02) and a marked familial autoimmunity background (OR 2·22, P = 0·01).Our study clearly shows that features of inflammation (pruritus)/autoimmunity (halo naevi, thyroid antibodies) are strongly linked to NSV, together with a familial background of vitiligo and autoimmunity.

Les melanomes de la muqueuse buccale representent 0,2 a 8% de tous les melanomes, 0,5% des tumeurs malignes de la cavite buccale et 48% des melanomes de la muqueuse bucco-nasale (Hicks et Flaitz 2000). La localisation la plus frequente (80% des cas) est le palais dur et la gencive maxillaire (Ebenezer 2006). Malgre leur rarete, ces lesions doivent etre evoquees dans le diagnostic differentiel des lesions pigmentees de la muqueuse buccale. L’objectif de ce travail est de presenter deux nouveaux cas de melanome de la muqueuse buccale dont le diagnostic etait evident dans un cas et retarde pour l’autre en raison d’une confusion avec une pigmentation exogene. Le premier cas concernait un homme de race blanche, de 68 ans, atteint d’un melanome de la cloison nasale gauche (2 cm de diametre), traite en 2001 par exerese chirurgicale et radiotherapie adjuvante. En aout 2007, est apparue une tumeur pigmentee de la levre superieure droite et de la gencive adjacente, associee a une amputation du champ visuel. Le diagnostic de recidive de melanome cliniquement evident, a ete confirme par la biopsie. La TEP-TDM a mis en evidence une hyperfixation en regard du maxillaire et du plancher buccal anterieur. La TDM a montre un micronodule pulmonaire droit de moins d'1 cm de diametre. Le patient a ete traite par chimiotherapie (dacarbazine 1000mg.m). Apres 11 cures sur 11 mois, le melanome semblait stable. Le deuxieme cas concernait une femme de race blanche, de 63 ans, sans antecedents familiaux de melanome. La patiente a consulte son chirurgien dentiste en juin 2007 pour une douleur dentaire sur l’arcade superieure gauche. L’examen clinique mettait en evidence un amalgame ancien sur 26 avec a une pigmentation gingivale en regard mesurant 2 cm de long. Un tatouage lie a l’amalgame a ete suspecte. Le chirurgien-dentiste a effectue les traitements conservateurs et prothetiques sur 25 et 26. Cependant, apres plusieurs mois, devant la persistance de la lesion et l’apparition d’une adenopathie homolaterale, une biopsie gingivale et ganglionnaire a ete realisee. L’examen anatomopathologique a confirme le diagnostic de melanome. Le bilan d’extension a revele des metastases hepatiques et pulmonaires. Apres 3 cures de dacarbazine, la patiente est decedee en avril 2008. Les melanomes buccaux sont des tumeurs agressives dont la physiopathologie est mal connue. Le diagnostic de melanome est facile lorsqu’il s’agit d’une localisation secondaire et que la tumeur primitive est connue (cas 1), par contre le diagnostic d'une tumeur primitive est plus difficile (cas 2). Les criteres diagnostiques ABCDE (Asymetrie, Bords irreguliers, Couleur foncee, Diametre superieur a 6 mm, Evolution) proposes pour les melanomes cutanes, peuvent aussi constituer une aide pour le diagnostic des melanomes buccaux (Auluck 2008). L’exerese de toute lesion pigmentee non etiquetee ou suspecte doit etre systematiquement realisee en vue d’un examen anatomopathologique. Si la prevention est primordiale pour les melanomes cutanes, malheureusement celle-ci est impossible pour les melanomes muqueux car les facteurs de risque sont inconnus. Aussi, la formation des professionnels de sante a l’examen systematique la muqueuse buccale et a la reconnaissance des lesions a risque est indispensable si l’on veut modifier le pronostic des melanomes buccaux primitifs. 54eme Congres de la SFMBCB, 03008 (2011) DOI: 10.1051/sfmbcb/20115403008 © Owned by the authors, published by EDP Sciences, 2011

based on consensual recommendations, surgery remains the standard treatment for curable lung metastases. In this setting, radiofrequency ablation of limited metastases has been described in numerous studies in recent years. We report herein two patients presenting with low-burden lung metastases from malignant melanoma treated by radiofrequency.two patients presented with one to two limited burden lung metastases from malignant melanoma, respectively. Both patients received neoadjuvant chemotherapy leading to disease stabilization, after which the lung metastases were treated by radiofrequency. Both patients had complete remission following radiofrequency, with 12 and 21 months follow-up respectively.surgical treatment of solitary or scant pulmonary metastases from melanoma has proved its efficacy with a gain in overall and disease-free survival. Nevertheless, this treatment cannot be proposed in patients with contraindications for anaesthesia or compromised pulmonary function. In this population, radiofrequency ablation appears to offer a potentially valuable alternative to surgery. Additionally, the related morbidity and duration of hospitalisation associated with radiofrequency seemed to be improved. As seen in these two cases, the efficacy and increased survival achieved with radiofrequency appears comparable to those obtained through surgery.

Microvenular hemangioma belongs to the group of acquired vascular tumors. It is important to differentiate such lesions because of their prognostic and nosologic consequences. We report a case of microvenular hemangioma.A 31 year-old man presented with a 3 cm erythematous and asymptomatic nodule of the abdomen, which had grown for 2 months. Histopathology showed the irregular dermal proliferation of small vessels, composed of capillaries and venules, without atypia. No relapse was noted 6 months after complete exeresis.Microvenular hemangioma is a recently described vascular tumor. The first three cases were reported in 1989, with the denomination of "microcapillar hemangioma". Twenty-one further cases have been reported since 1991. We discuss the typical clinical and histological characteristics of this lesion and present criteria permitting the differential diagnosis with other vascular neoplasms. Dermatologists should be aware of this lesion, notably for the differential diagnosis with early onset Kaposi's disease.

The cutaneous signs of toxicomania are better known since the last two decades. We describe an original case of segmental necrotic and chronic livedo of the right arm associated with cocaine use.A 31 year-old intravenous cocaine user presented a necrotic and painful livedo of the right arm. Two years before referral, localized recurrent edema was noted without general involvement. No local (infectious, vascular) or general (dysimmunity, coagulation abnormalities) aetiologies could be found. An amorphic substance composed of mineral bodies was found in the dermis, without damage to the vessels. Dramatic improvement was obtained with simple supportive care, while intravenous injections were stopped.The vasoconstrictive properties of cocaine have been demonstrated in Raynaud's phenomenon, ischemia, focal necrosis of the extremities, and also in liver and kidney disorders. This drug has pharmacological effects on endothelial cells and coagulation, explaining the arterial and venous thrombosis reported. The foreign bodies found in our patient probably came from an intraarterial injection. The progressive and chronic evolution, without any evidence of thrombosis at the time of examination, are suggestive of the vasoconstrictive action of chronic dermal deposits.

We report a case of arrhythmogenic right ventricular cardiomyopathy revealed by sudden death during exercise in a 13-year-old patient. Postmortem diagnosis was made on multiple tissue samples taken from right ventricular free wall, showing light adipous infiltration of the myocardium at gross examination. Arrhythmogenic right ventricular cardiomyopathy is histologically characterized by fibro-fatty replacement of right ventricular myocardium. Left ventricular involvement may be observed. Diagnosis at an early stage is often difficult. Etiology remains unknown. Since familial occurrence has been documented, postmortem identification is useful for the other members of the family.

Journal de Gynecologie Obstetrique et Biologie de la Reproduction - Vol. 41 - N° 2 - p. 206