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Anogenital granulomatosis

Authors: Dr Jacqueline Chen, Dermatology Resident; Dr Rachel Manifold, Dermatologist, Flinders Medical Center, Australia. Copy edited by Gus Mitchell. April 2022


Anogenital granulomatosis — codes and concepts
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What is anogenital granulomatosis?

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.

Who gets anogenital granulomatosis?

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.

What causes anogenital granulomatosis?

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.

What are the clinical features of anogenital granulomatosis? 

Most commonly presents as:

  • Chronic penile, scrotal, vulval, or anoperineal swelling
  • With or without erythema.

Other clinical features include:

  • Fissures, perianal tags and ulcers
  • Difficulties with urinary flow and sexual intercourse.

What are the complications of anogenital granulomatosis?

  • Recurrent cellulitis.
  • Impact on mental health and quality of life.
  • Urinary flow and sexual difficulties.

How is anogenital granulomatosis diagnosed?

Anogenital granulomatosis is usually diagnosed clinically.

A biopsy is helpful to confirm the diagnosis. The characteristic features seen in biopsy include:

  • Scattered, non-caseating epithelioid cell granulomas
  • Lymphoedema and granulomas containing epithelioid cells, multinucleated giant cells, and lymphocytes.

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.

What is the differential diagnosis for anogenital granulomatosis?

Non-infectious causes

Infective causes

What is the treatment for anogenital granulomatosis?

Treatment is challenging and based on limited case studies.

General measures

  • Compression shorts for support and to reduce discomfort

Specific treatment

 

Bibliography

  • Alexakis C, Gordon K, et al. Anogenital granulomatosis and Chron’s disease: a case series of males presenting with genital lymphoedema. J Chrons Colitis 2017; 11(4):454–9. DOI:10.1093/ecco-jcc/jjw173. Journal
  • Honap S, Meade S, Spencer A, et al. Anogenital Crohn's disease and granulomatosis: a systematic review of epidemiology, clinical manifestations, and treatment. J Crohns Colitis. 2021;jjab211. doi:10.1093/ecco-jcc/jjab211. Journal
  • Saracino A, Gordon K, Ffolkes L, Mortimer P. Intralymphatic granulomas in lymphedema secondary to anogenital granulomatosis. Aust J Dermatol. 2012;54(3). DOI: 10.1111/j.1440-0960.2012.00923.x. Journal
  • Van de Scheur MR, Van der Waal RIF, van der Waal TJ Stoff,  Van Deventer SJH. Anogenital granulomatosis: the counterpart of orofacial granulomatosis. JEADV 2003; 27: 184–9. DOI: 10.1046/j.1468-3083.2003.00573.x. Journal
  • Villar M, Petit G, Gurra A, Vanaclocha F. Anogenital granulomatosis. Actas Dermo-Sifiliogr. 2012; 103(1):76–9. DOI: 10.1016/j.adengl.2011.05.007. PubMed

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