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Τετάρτη 6 Ιουνίου 2018

Vulvite granulomateuse de type Miescher

Publication date: Available online 6 June 2018
Source:Annales de Dermatologie et de Vénéréologie
Author(s): K. Alhazmi, S. Berville, M. Moyal-Barracco, F. Plantier
IntroductionLa chéilite de Miescher, isolée ou dans le cadre d'un syndrome de Melkerssohn-Rosenthal, est une inflammation granulomateuse rare de cause inconnue dont il existe un équivalent génital encore plus rare. Nous en décrivons un cas, avec son évolution sur douze années.ObservationUne femme de 27 ans présentait des poussées d'œdème vulvaire unilatéral, régressives au début puis aboutissant au fil du temps à des tuméfactions permanentes, vulvaire et périnéale. Les prélèvements histologiques montraient des granulomes histiocytaires épithélioïdes, si discrets qu'ils étaient passés inaperçus au début de l'évolution. L'œdème demeurait un signe isolé pendant douze ans et deux bilans digestifs à la recherche d'une maladie de Crohn étaient négatifs, permettant d'écarter ce diagnostic.DiscussionLa vulvite granulomateuse de type Miescher est un diagnostic d'élimination, essentiellement de la maladie de Crohn. Notre observation illustre la difficulté du diagnostic de cette pathologie rare et les incertitudes concernant sa physiopathologie. Le diagnostic impose de savoir répéter les prélèvements et pratiquer des biopsies profondes avec des niveaux de coupes répétés pour identifier les granulomes paravasculaires caractéristiques, parfois très discrets.BackgroundMiescher's cheilitis, whether occurring alone or as part of Melkersson-Rosenthal syndrome, is a rare type of granulomatous inflammation of unknown cause with an even rarer genital equivalent. Herein, we describe a case of the latter condition developing over a 12-year period.Patients and methodsA 27-year-old woman presented episodes of unilateral vulvar oedema, which initially regressed but resulted over time in permanent vulvar and perineal tumefaction. Histology revealed epithelioid histocytic granulomas so mild that they were not noticed at the start of the disease. The oedema remained the sole sign for 12years and two gastrointestinal screening tests for Crohn's disease proved negative, diagnosis of the latter condition was ruled out.DiscussionMiescher's granulomatous vulvitis requires differential diagnosis, essentially with regard to Crohn's disease. Our case illustrates the difficulty in diagnosing this rare disease as well as the uncertainties surrounding its physiopathology. Diagnosis rests upon repeated sampling and biopsies with repeated levels of sections in order to identify the characteristic perivascular granulomas, which may be very mild.



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