Lipschütz genital ulcer revisited: is juvenile gangrenous vasculitis of the scrotum the male counterpart?

Abstract

Since its first description as ulcus vulvae acutum by Benjamin Lipschütz in 1912, the etiopathogenesis of this peculiar genital ulcer remains incompletely understood. In his original description, two different types of genital ulcers were observed and proposed, which were not precisely defined and distinguished in most subsequent reports. The first type is characterized by acute excruciating genital ulcers of first‐time onset with self‐limited non‐recurrent course in association with gravely symptomatic systemic infections, in which a primary Epstein–Barr virus (EBV) infection is later identified to be probably the most common aetiology. The second type of ulcer usually refers to little painful ulcers of unknown etiopathogenesis in the absence of fever or chills and with a slow torpid progression and recurrent nature. Differentiation from idiopathic aphthous ulcers is unclear. The changes of the cervicovaginal microbiota and microbiome in diseased state deserve further clarification. Acute genital ulcers associated with primary EBV infection in women have drawn attention since 1970s, while the corresponding penile ulcers in men were already known in 1950s. First presented in 1973, juvenile gangrenous vasculitis of the scrotum with an acute painful scrotal ulcer preceded by symptomatic pharyngeal infections can be considered as the male counterpart of ulcus vulvae acutum, and the future clinical survey should include primary EBV infection.

Juvenile gangrenous vasculitis of the scrotum: A rare cause of genital ulcer

“…This presentation is particularly similar to Lipschutz ulcers in women, with a suggestion that these may share the same pathogenic mechanism 8,9 …”

“…[2][3][4][5][6][7] This presentation is particularly similar to Lipschutz ulcers in women, with a suggestion that these may share the same pathogenic mechanism. 8,9 Due to the clinicopathological features, consideration of a variant of pyoderma gangrenosum was proposed 4 but the early age onset, unusual localization, histology of vasculitis, rapid favourable treatment response and benign lenient disease course in the absence of underlying diseases are different to the manifestations of classical pyoderma gangrenosum. 8…”

Juvenile Gangrenous Vasculitis of the Scrotum: A Case to Consider

“…During admission, the patient is evaluated by the Dermatology Service, which performs a skin biopsy of the lesion, observing areas of ulceration with vasculitis and fibrin thrombi in the pathological anatomy, as well as an acute inflammatory infiltrate with neutrophilic interventions or directed therapies except for good local hygiene with daily cures [1,6]. Surgical debridement although it is essential when it comes to necrotizing fasciitis, in this entity it is completely unnecessary and can contribute morbidity to the condition.…”

Juvenile gangrenous vasculitis of the scrotum – a diagnosis not to forget

“…It is accompanied by elevation of acute phase reactants 2. This acute presentation is often distressing and alarming for both patients and clinicians.The histopathologic features comprise mostly neutrophilic dermal infiltrate, fibrinoid necrosis of small blood vessel walls, and absence of leukocytoclastic vasculitis 5. Once JGVS histopathology resembled pyoderma gangrenosum (PG), it was questioned if they could represent the same entity, 2 although their clinical courses are very distinct 4.…”

“…Once JGVS histopathology resembled pyoderma gangrenosum (PG), it was questioned if they could represent the same entity, 2 although their clinical courses are very distinct 4. Very recently, Chen W et al proposed that the overlap between JGVS and ulcus acutum vulvae renders possible that they may be counterparts of the same type of Lipsch€ utz genital ulcer 5. The differential diagnosis of JGVS includes numerous ulcerative scrotal diseases, such as sexually transmitted…”