Recurrent reactive non-sexually related acute genital ulcers: a risk factor for Behcet’s disease?

Recurrent reactive non-sexually related acute genital ulcers: a risk factor for Behcet’s disease?



acute genital ulcers, non-sexually transmitted genital ulcers, Lipschütz ulcers, ulcus vulvae acutum, recurrent aphtous ulcers, complex aphthosis, Behcet’s disease, adolescents, children


Background and aim: Lipschutz ulcers (LU) are idiopathic genital lesions characterized by the sudden appearance of painful, usually symmetric vulvar ulcers, typically occurring in sexually inactive adolescents. LU is a diagnosis of exclusion. As these lesions heal spontaneously, in the absence of tissue scarring, the therapy is mainly symptomatic and focuses on pain relief. Recurrence of LU associated with oral ulcers describes the clinical picture of complex aphthosis, which belongs to Behçet’s disease (BD) pathological spectrum. Our work aims to analyze the correct diagnostic approach to recurrent aphthous, focusing on the importance of a multidisciplinary assessment and immunogenetic investigation to identify the subjects at risk of progression towards BD.  

Methods: We present the case of a 12-year-old non sexually active Italian girl who was diagnosed with LU. After 15 months, she presented recurrent reactive non sexually related acute genital ulcer associated with a history of oral aphthous. According to clinical features and anamnesis, complex aphthosis was diagnosed. For diagnostic purposes, she underwent an immunogenetic analysis that showed HLA-B51 positivity.

Results: In the absence of clinical and laboratory criteria to define the risk of progression of complex aphtosis towards BD, we think that besides a strict follow-up, in pediatric patients with a suggestive clinical history, it is crucial to adopt a multidisciplinary approach, comprehensive of HLA investigation, in order to guarantee an early diagnosis and a prompt therapeutic intervention.

Conclusions: In children and adolescents with genital ulcers, it is essential to consider all the possible differential diagnoses to undertake a timely and correct course of treatment.





Farhi D, Wendling J, Molinari E, et al. Non-sexually related acute genital ulcers in 13 pubertal girls: a clinical and microbiological study. Arch Dermatol. 2009;145(01):38–45.

Lai, K., Lambert, E. & Mercurio, M. G. Aphthous Vulvar Ulcers in Adolescent Girls: Case Report and Review of the Literature. J. Cutan. Med. Surg. 2010;14, 33–7

Huppert JS. Lipschutz ulcers: evaluation and management of acute genital ulcers in women. Dermatol Ther. 2010;23(5):533-40.

Lehman JS, Bruce AJ, Wetter DA, et al. Reactive nonsexually related acute genital ulcers: review of cases evaluated at Mayo Clinic. J Am Acad Dermatol. 2010;63(1):44-51.

Arellano J, Fuentes E, Moreno P, et al. Ulcer of Lipschütz, a diagnosis to consider in the pediatric population. Arch Argent Pediatr. 2019;117(3):e305-e8.

Eizaguirre FJ, Lucea L, Artola E, et al. Acute genital ulcer unrelated to a sexually transmitted disease. An Pediatr (Barc). 2012;76(3):170-2.

Rosman IS, Berk DR, Bayliss SJ, et al. Acute genital ulcers in nonsexually active young girls: case series, review of the literature, and evaluation and management recommendations. Pediatr Dermatol. 2012;29(2):147-53.

Keogan MT. Clinical Immunology Review Series: an approach to the patient with recurrent orogenital ulceration, including Behçet's syndrome. Clin Exp Immunol. 2009;156(1):1-11.

Koné-Paut I. Behçet's disease in children, an overview. Pediatr Rheumatol Online J. 2016;14(1):10.

Costagliola G, Cappelli S, Consolini R. Behçet's Disease in Children: Diagnostic and Management Challenges. Ther Clin Risk Manag. 2020;16:495-507.

Pereira DAG, Teixeira EPP, Lopes ACM, et al. Lipschütz Ulcer: An Unusual Diagnosis that Should Not be Neglected. Rev Bras Ginecol Obstet. 2021;43(5):414-6.

Maldini C, Lavalley MP, Cheminant M, et al. Relationships of HLA-B51 or B5 genotype with Behcet's disease clinical characteristics: systematic review and meta-analyses of observational studies. Rheumatology (Oxford). 2012;51(5):887-900.




How to Cite

Brambilla I, Moiraghi A, Guarracino C, Pistone C, Tondina E, Riccipetitoni G, et al. Recurrent reactive non-sexually related acute genital ulcers: a risk factor for Behcet’s disease? . Acta Biomed [Internet]. 2022 Jun. 6 [cited 2024 Jul. 22];93(S3):e2022196. Available from: