SORA

Advancing, promoting and sharing knowledge of health through excellence in teaching, clinical practice and research into the prevention and treatment of illness

Ano-genital Granulomatosis and Crohn's Disease: A Case Series of Males Presenting with Genital Lymphoedema.

Alexakis, C; Gordon, K; Mellor, R; Chong, H; Mortimer, P; Pollok, R (2017) Ano-genital Granulomatosis and Crohn's Disease: A Case Series of Males Presenting with Genital Lymphoedema. J Crohns Colitis, 11 (4). pp. 454-459. ISSN 1876-4479 https://doi.org/10.1093/ecco-jcc/jjw173
SGUL Authors: Mortimer, Peter Sydney Pollok, Richard Charles G

[img]
Preview
PDF Accepted Version
Available under License ["licenses_description_publisher" not defined].

Download (1MB) | Preview

Abstract

Background and Aims: Ano-genital granulomatosis is a rare chronic granulomatous condition of the skin that causes lymphoedema of the external genitalia. There is a reported association with Crohn's disease. Mechanisms of disease and optimal methods of treatment are poorly understood. Methods: A retrospective casenote review of 25 male patients with ano-genital granulomatosis presenting with genital lymphoedema was performed to determine the clinical and histopathological features of this condition and its relationship to intestinal Crohn's disease. Results: A combination of penile and scrotal oedema was reported at presentation in 80% of patients; 40% of patients had associated intestinal Crohn's disease. The average time from symptom onset to diagnosis was 52.7 months. Half of cutaneous biopsies contained non-caseating granulomas and 14% contained intralymphatic granulomas. In all, 72% of patients responded to oral steroids initially but recurrence was common. Complete or partial response was achieved in 60% of patients treated with azathioprine. Three of six patients responded to anti-tumour necrosis factor [TNF] therapy. A small proportion of patients required circumcision or de-bulking surgery for more debilitating disease. Conclusions: Ano-genital granulomatosis is a rare condition that presents with genital lymphoedema, and there is frequently a protracted delay in diagnosis. There is a very strong association with intestinal Crohn's disease. Genital lymphoedema associated with gastrointestinal symptoms should prompt careful evaluation to exclude both ano-genital granulomatosis and Crohn's disease.

Item Type: Article
Additional Information: This is a pre-copyedited, author-produced version of an article accepted for publication in Journal of Crohn's and Colitis following peer review. The version of record Christopher Alexakis, Kristiana Gordon, Russell Mellor, Heung Chong, Peter Mortimer, Richard Pollok; Ano-genital Granulomatosis and Crohn’s Disease: A Case Series of Males Presenting with Genital Lymphoedema, Journal of Crohn's and Colitis, Volume 11, Issue 4, 1 April 2017, Pages 454–459 is available online at: https://doi.org/10.1093/ecco-jcc/jjw173
Keywords: Ano-genital granulomatosis, Crohn’s disease, lymphoedema, Adolescent, Adult, Aged, Anus Diseases, Azathioprine, Child, Crohn Disease, Genital Diseases, Male, Granuloma, Humans, Immunosuppressive Agents, Lymphedema, Male, Middle Aged, Penile Diseases, Retrospective Studies, Scrotum, Tumor Necrosis Factor-alpha, Young Adult, Ano-genital granulomatosis, Crohn's disease, lymphoedema, Ano-genital granulomatosis, Crohn’s disease, lymphoedema, Gastroenterology & Hepatology, 1103 Clinical Sciences
SGUL Research Institute / Research Centre: Academic Structure > Infection and Immunity Research Institute (INII)
Academic Structure > Molecular and Clinical Sciences Research Institute (MCS)
Academic Structure > Molecular and Clinical Sciences Research Institute (MCS) > Cell Sciences (INCCCS)
Journal or Publication Title: J Crohns Colitis
ISSN: 1876-4479
Language: eng
Dates:
DateEvent
1 April 2017Published
18 October 2016Published Online
27 September 2016Accepted
PubMed ID: 27683802
Web of Science ID: WOS:000398161300008
Go to PubMed abstract
URI: http://openaccess.sgul.ac.uk/id/eprint/109410
Publisher's version: https://doi.org/10.1093/ecco-jcc/jjw173

Actions (login required)

Edit Item Edit Item