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Résumé: Le prurit anal est une affection fréquente qui peut altérer de manière significative la qualité de vie des patients. Sa prise en charge est complexe du fait d'un éventail étiologique large. Cependant, le prurit anal primaire (essentiel ou idiopathique) en est la cause la plus fréquente. Ce diagnostic est retenu en l'absence de cause secondaire évidente. Sa prise en charge repose essentiellement sur des mesures hygiéno-diététiques associées à des traitements topiques ayant pour but d'assécher et de protéger la peau mais également de rompre le cercle vicieux du prurit anal grâce aux dermocorticoïdes. Les causes secondaires sont multiples, dominées par les maladies infectieuses qui peuvent être suspectées d'emblée en cas d'évolution généralement récente, d'un aspect clinique évocateur et, si besoin, par des prélèvements locaux positifs. Ceci étant dit, un traitement antiparasitaire d'épreuve peut être prescrit d'emblée. Les maladies dermatologiques sont également fréquentes et dominées par le psoriasis inversé mais aussi l'eczéma de contact ou le lichen plan ou scléreux. Leur diagnostic repose sur l'anamnèse et, si besoin, l'analyse histologique. Enfin, un prélèvement à visée histologique est également indiqué en cas d'échec des traitements de première ligne et/ou en cas d'évolution récidivante afin de ne pas méconnaître une affection tumorale comme la maladie de Bowen (High Grade Squamous Intraepithelial Lesion) ou la maladie de Paget qui nécessitent une prise en charge spécifique. Anal pruritus is a common condition that can significantly impair patients' quality of life. Its management is complex due to a wide range of etiologies. However, primary (essential or idiopathic) anal pruritus is the most common cause. This diagnosis is made in the absence of an obvious secondary cause. Its management is essentially based on hygienic and dietary measures associated with topical treatments aimed at drying and protecting the skin, but also at breaking the vicious circle of anal pruritus with dermocorticoids. Secondary causes are multiple, dominated by infectious pathologies which can be suspected from the outset in the case of a generally recent evolution, an evocative clinical aspect and, if necessary, on positive local samples. This being said, a trial antiparasitic treatment may be prescribed from the outset. Dermatological pathologies are also frequent and dominated by inverted psoriasis but also contact eczema or lichen planus or sclerosus. Their diagnosis is based on anamnesis and, if necessary, histological analysis. Finally, a histological sample is also indicated in the event of failure of first-line treatments and/or in the event of recurrent evolution in order not to ignore a tumoral affection such as Bowen's disease (High Grade Squamous Intraepithelial Lesion) or Paget's disease, which require a specific management [ABSTRACT FROM AUTHOR]

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Résumé: La maladie de Paget est une maladie osseuse chronique touchant 3 % de la population caucasienne après 55 ans. La cause de cette pathologie reste inconnue, mais une contribution génétique est démontrée dans certaines formes familiales. Ces formes suivent un mode de transmission autosomique dominant avec pénétrance incomplète. Une hétérogénéité génétique est démontrée avec huit loci décrits. Un premier gène est connu, Sequestosome1 (SQSTM1), situé sur le bras long du chromosome 5 (5q35-qter). Neuf mutations de ce gène ont déjà été rapportées dans la maladie de Paget. Ces mutations siègent dans les exons 7 et 8 du gène, codant pour le domaine très conservé de liaison à l''ubiquitine. Le test génétique des mutations de SQSTM1 est recommandé chez les patients atteints de maladie de Paget. Vu la fréquence des formes cliniquement asymptomatiques, la notion familiale fait souvent défaut chez les patients porteurs d''une mutation, si bien que le test est recommandé y compris en l''absence d''antécédents familiaux de maladie de Paget. L''identification des patients porteurs d''une mutation de SQSTM1 (près de 10 % en France) permet de proposer un conseil génétique chez les apparentés en vue d''un dépistage précoce de la maladie chez les porteurs, dans le but de leur éviter la révélation de la maladie de Paget par une complication. [Copyright &y& Elsevier]

Paget's disease of the femur and tibia causes severe morbidity and mortality in the elderly population. It weakens and deforms the bone, causing significant disability. Deformities have been corrected with various methods, mostly using external fixation. In this study, we have utilised locked intramedullary nails after osteotomy to rectify these bone deformities. A number of patients were studied and followed up using a standard method to document outcome. The aim of this investigation was to determine the clinical effectiveness of internal fixation with interlocking nails for the treatment of deformities of pagetoid bones. The safety and effectiveness in terms of function, pain relief, and cosmetic results were determined after using internal fixation to correct deformed tibiae and femora. The goal of treatment was to restore a normal axis in the limb and allow early weight bearing, preventing mineral loss and additional bone weakening. The operative procedures are described and the pre- and post-operative appearance of the lower limb bones shown. The advantages of the procedure as compared to other methods of fixation are discussed. [ABSTRACT FROM AUTHOR]

Le nombre de cas d’ostéonécroses des mâchoires est en constante augmentation. Certaines surviennent chez des patients traités par bisphosphonates oraux pour ostéoporose ou maladie de Paget, mais la plupart concerne des patients traités par bisphosphonates par voie intraveineuse dans le cadre d’un traitement oncologique. La prise en charge est pluridisciplinaire. [ABSTRACT FROM AUTHOR]

Abstract: Paget''s disease of the vulva is difficult to diagnose, as far as the extent of this lesion is concerned, since it is often coupled with invasive adenocarcinoma of the vulva. Thus, we present two cases of Paget''s disease of the vulva, with use of sentinel lymph node analysis— a non-validated technique in this very context. Evaluation of sentinel lymph node may be useful in case of micro-invasive Paget''s disease. [Copyright &y& Elsevier]

Background: Paget's disease of the breast is rare, even more so when it occurs in a supernumerary breast since diagnosis is delayed due to lack of exploration and ignorance of the pathology of ectopic breast. Based on an original clinical case, we provide a clinical update concerning ectopic breast and ectopic carcinoma, including Paget's disease., Patients and Methods: A 44-year-old woman had complained of pain in the area of her supernumerary breast for several months. The ultrasound examination was normal but the unexplained symptoms led to performance of an excision biopsy of the lesion. This showed strictly intra-epidermal Paget's disease measuring 2mm, without any underlying carcinoma. A wider excision was performed. Follow-up at one year showed no recurrence., Discussion: An ectopic breast, present in 6 % of the population, is often ignored, despite being subject to the same diseases as anatomical breasts: it is at the origin of 0.6 % of breast cancers. Paget's disease is a rare in situ adenocarcinoma comprising fewer than 3 % of cases of breast cancer. It is extremely rare in ectopic breast. Ours is only the fourth reported case in the literature. This case is original because of the lack of objective clinical signs. Subjective symptoms such as pain, tingling, burning sensation and pruritus related to the supernumerary breast must be taken into consideration in order to enable early diagnosis of Paget's disease. Exploration by ultrasound and mammography generally appears flawed. Breast MRI may be useful but is not performed routinely. A promising future approach may consist of confocal microscopy screening of all ectopic breasts with areola or nipple patches, since this method enables the visualization of Paget cells and their organization., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)