Genital Granulomatosis in Male and Female Patients With Crohn's Disease: Clinical Presentation and Treatment Outcomes
Por:
Dederichs, F, Iesalnieks, I, Sladek, M, Tzivinikos, C, Hansen, R, Munoz, C, Pavli, P, Cavicchi, M, Abitbol, V, Rahier, JF, Vavricka, S, Katsanos, K and Domenech, E
Publicada:
1 feb 2018
Resumen:
Background: Genital granulomatosis [GG] is a metastatic form of Crohn's disease [CD], characterised by granulomatous inflammation of the genital skin without contact with the gastrointestinal tract. Little is known about GG, as most publications are case reports or small series, and only sporadic in male cases.
Methods and Aims: Cases of GG were retrospectively collected through the Collaborative Network For Exceptionally Rare case reports project of the European Crohn ' s and Colitis Organisation.
Results: A total of 43 patients [9 males, 34 females] were diagnosed as having GG, mostly as oedema and/or ulcers. Histological confirmation of granulomas was obtained in 70% of the cases. CD location was colonic or ileocolonic in 97% and perianal disease was documented in 57%. There was no significant difference between males and females in CD phenotype or genital lesions. GG was the first manifestation of inflammatory bowel disease [IBD] in one-third of the patients; these patients were younger at the time of GG occurrence and they all were non-smokers. GG occurred in the absence of gastrointestinal disease activity in 30% of the cases. Ten out of 11 patients [91%] responded to systemic corticosteroid treatment, 5/9 patients responded to immunomodulators, and 9/11 patients responded to anti-tumour necrosis factor alpha [TNF-alpha] agents.
Conclusions: GG is a rare extraintestinal manifestation of CD. It mainly occurs among women, in the setting of colonic involvement of CD, and perianal disease is often associated. Most cases are successfully managed with systemic corticosteroids or anti-TNF agents.
Filiaciones:
Dederichs, F:
Kath Krankenhaus Hagen, Hagen, Germany
Iesalnieks, I:
Klinikum Bogenhausen, Munich, Germany
Sladek, M:
Jagiellonian Univ, Med Coll, Krakow, Poland
Tzivinikos, C:
Royal London Hosp, London, England
Hansen, R:
Royal Hosp Children, Glasgow, Lanark, Scotland
Munoz, C:
Villafranca Hosp Univ Basurto, Bilbao, Spain
Pavli, P:
Canberra Hosp, Canberra, ACT, Australia
Cavicchi, M:
Cabinet Med Creteil, Paris, France
Abitbol, V:
Cochin Hosp, Paris, France
Rahier, JF:
CHU Dinant Godinne, Yvoir, Belgium
Vavricka, S:
Triemli Hosp, Zurich, Switzerland
Katsanos, K:
Univ & Med Sch Ioannina, Ioannina, Greece
:
Hosp Badalona Germans Trias & Pujol, Badalona, Spain
CIBEREHD, Madrid, Spain
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