Ulcers in the genital area are usually caused by sexually transmitted infections (STIs). These include herpes, syphilis, and chancroid. However, they might also be caused by inflammatory diseases, trauma, autoimmune bullous disorders or cutaneous malignancies. In young women, Lipschütz Ulcer, or ulcus vulvae acutum, is a rare and probably underdiagnosed entity with unknown aetiology, although recent reports have associated it with the Epstein-Barr virus.

Diagnosis of most of these conditions is established by exclusion after ruling out sexually transmitted diseases, and finally with the help of histopathology. An exception is Behçet’s disease (BD), a multisystem chronic inflammatory disorder, diagnosed based on the established diagnostic criteria and/or PFAPA, PAPA and TRAPS syndromes which are diagnosed by exclusion and genetic means. A complex genetic background leading to activation of the innate immune system and particularly inflammasomes through auto- or environmental antigens is presumably the causative.

Accordingly, apart from corticosteroids and colchicine, anti-TNF-α therapy may be useful in conventional therapy-resistant refractory and severe BD as well in patients with PFAPA syndrome. With this abstract we would like to present and discuss our experience on diagnosis as well as treatment of “Difficult to Diagnosis and Treat” patients with genital ulcers from the DIAID of the Department of Dermatology at the Medical University of Vienna.

Beside STIs as the cause genital ulcers, autoinflammatory disorders should be also considered. Here, novel approaches such as anti-TNF-a and IL-1R blockers are promising therapeutic strategies and warrant multicenter clinical studies.