Granulomatous lymphangitis of the scrotum and penis

Westermark and Hendriksson 1979

Anogenital granulomatosis is a rare disease associated with genital swelling of the genitals (penis and vulva), which initially recurs and later persists, is firm and often painful (Makatsori M et al. 2013; Gordon KD et al. 2013). In this clinical picture there is a very strong association with a regional enterocolitis (Crohn's disease) in the intestine or a Melkersson-Rosenthal syndrome. The average time from onset of symptoms to diagnosis was 52.7 months in a collective of 25 patients.

Granulomatous lymphangitis of the genitals is probably not an independent clinical picture but an abortive variant of Melkersson-Rosenthal syndrome or a cutaneous manifestation of Crohn's disease (Alexakis C et al.2017). An associated Crohn's disease can be diagnosed in almost 50% of patients with granulomatous lymphangitis of the scrotum and penis.

In a collective of 25 patients, half of the skin biopsies contained non-cheating granulomas. In 14% of cases intralymphatic granulomas were detectable (Alexakis C et al.2017).

The majority of patients initially reacted positively to oral steroids. However, recurrences occurred regularly after discontinuation. Better effects were achieved with azathioprine or TNF-alpha blockers (Alexakis C et al.2017).

The histological pattern of granulomatous lymphangitis is most frequently associated with orofacial granulomatosis (granulomatous cheilitis and Melkersson-Rosenthal syndrome) and Crohn's disease.

1. Alexakis C et al (2017) Ano-genital Granulomatosis and Crohn's Disease: A Case Series of Males Presenting with Genital Lymphoedema. J Crohn's colitis 11:454-459.
2. Gordon KD et al (2013) Genital lymphoedema due to ano-genital granulomatosis. Int J STD AIDS 24:149-151.
3. Makatsori M et al (2013) Penile granulomatosis presenting as pseudoangioedema. Eur Ann Allergy Clin Immunol 45:111-112.
4. Murphy MJ et al (2001) Granulomatous lymphangitis of the scrotum and penis. Report of a case and review of the literature of genital swelling with sarcoidal granulomatous inflammation. J Cutan Pathol 28:419-424.
5. Westermark P et al (1979) Granulomatous inflammation of the vulva of the penis-a genital counterpart fo cheilitis granulomatosa. Dermatologica 158: 269-274