Juvenile gangrenous vasculitis of the scrotum (JGVS) is a rare entity with scant reports in the literature. The disease course, treatment, and prevalence have not been well described in the literature. It's hypothesized that JGVS is a variant of pyoderma gangrenosum or a male counterpart of Lipschütz ulcer. This review will analyze the current literature on JGVS and provide a current guide based on the best available data. The initial search of databases yielded 107 studies of which 14 pertained to the topic. The majority of the included studies were case reports (n = 9) reported in Spain. A total of 17 patients were included in the study. The mean age of patients was 22.45 years (range, 13-35 years). The majority of patients presented with multiple, acute, painful, well-circumscribed, round scrotal ulcerations. The majority of patients presented with flu-like symptoms. An increased level of awareness of JGVS diagnosis is now warranted among physicians. Despite the analogies with Lipschütz ulcer, we believe that JGVS is a distinct entity.
© 2022 the International Society of Dermatology.