Ano-genital Granulomatosis and Crohn's Disease: A Case Series of Males Presenting with Genital Lymphoedema.
OBJECTIVE
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.
Department of Gastroenterology, St George's University Hospital, London, UK.
Christopher
C
Alexakis
Department of Dermatology and Lymphoedema, St George's University Hospital, London, UK.
Kristiana
K
Gordon
Department of Dermatology and Lymphoedema, St George's University Hospital, London, UK.
Russell
R
Mellor
Department of Cellular Pathology, St George's University Hospital, London, UK.
Heung
H
Chong
Department of Dermatology and Lymphoedema, St George's University Hospital, London, UK.
Peter
P
Mortimer
Department of Gastroenterology, St George's University Hospital, London, UK.
Richard
R
Pollok
D000293:Adolescent; D000328:Adult; D000368:Aged; D001004:Anus Diseases / Q000150:complications* / Q000188:drug therapy; D001379:Azathioprine / Q000627:therapeutic use; D002648:Child; D003424:Crohn Disease / Q000150:complications* / Q000188:drug therapy; D005832:Genital Diseases, Male / Q000150:complications* / Q000188:drug therapy; D006099:Granuloma / Q000150:complications* / Q000188:drug therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008209:Lymphedema / Q000150:complications* / Q000188:drug therapy; D008297:Male; D008875:Middle Aged; D010409:Penile Diseases / Q000150:complications / Q000188:drug therapy; D012189:Retrospective Studies; D012611:Scrotum; D014409:Tumor Necrosis Factor-alpha / Q000037:antagonists & inhibitors; D055815:Young Adult
D007166:Immunosuppressive Agents; D014409:Tumor Necrosis Factor-alpha; D001379:Azathioprine
Ano-genital granulomatosis; Crohn’s disease; lymphoedema