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Introduction Demographics Causes Clinical features Complications Diagnosis Differential diagnoses Treatment
Anogenital granulomatosis (AGG) is a rare chronic inflammatory granulomatous disease of unknown aetiology. It is characterised by histological evidence of non-caseating granulomatous inflammation and can present with diffuse penile, scrotal, vulvar, or anoperineal swelling.
AGG is an uncommon condition that has been reported in both females and males of varying age groups from 1–79 years of age with the majority between 20–35 years of age. In 38% of cases, AGG can be associated with an underlying systemic condition such as Crohn disease or sarcoidosis. Associated Crohn disease may be symptomatically silent, or there may be a lag of several years between the appearance of genital swelling or lesions, and objective evidence of associated inflammatory bowel disease.
The cause of anogenital granulomatosis is unknown. It is characterised by granulomatous genital or anoperineal inflammation and lymphoedema. Intralymphatic granulomas have been noted to cause partial or complete occlusion of the drainage of lymphatics, subsequently causing lymphoedema.
Most commonly presents as:
Other clinical features include:
Anogenital granulomatosis is usually diagnosed clinically.
A biopsy is helpful to confirm the diagnosis. The characteristic features seen in biopsy include:
Further investigations, such as serum angiotensin-converting enzyme (ACE) and chest radiograph to exclude sarcoidosis can be conducted. Colonoscopy and faecal calprotectin to exclude Chron disease should be considered for patients with gastrointestinal symptoms.
There are reported cases of Crohn disease developing years after symptoms started, therefore long-term follow-up is warranted.
Treatment is challenging and based on limited case studies.
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