(81) Non-infectious genital ulcer as a sign of a systemic disease

Background: We report a case of an 11-year-old girl without relevant medical history, who had recurrent episodes of painful genital ulcers. Laboratories did not show any evidence of infection, so a systemic disease was suspected.

Case: An 11-year-old patient without medical comorbidities. was brought to the gynecological emergency room due to painful vulvar ulcers, without fever or other symptoms. No suggestive history of sexual abuse. Physical examination revealed 4 vulvar ulcers, the largest approximately 20 mm in diameter, with fibrin and an erythematous edge. Due to the pain it was decided to hospitalize her under initial suspicion of an herpes-simplex-virus ulcer. Laboratories were requested, including a test for herpes-simplex-virus, a sexually transmitted diseases panel test and immunoglobulins for cytomegalovirus and Epstein Barr virus, which were negative. A sample was sent for biopsy and cultures. Cultures were negative. She progressed favorably and was discharged after 4 days. She went for a check-up with a children's gynecologist 10 days later. Anamnesis revealed a history of recurrent orogenital aphthosis in the family (her mother and grandmother). No history of ophthalmological, neurological or cutaneous symptoms. On examination there were 2 vulvar ulcers without erythema or pain. Biopsy reported nonspecific inflammatory changes. She was referred to a pediatric gastroenterologist and rheumatologist, where no elements of inflammatory bowel disease were found, and calprotectin levels were negative. Rheumatologist requested antibodies, complement and immunoglobulins levels.
After 2 months she developed new painful aphthosis in vulva and oral mucosa, no other symptoms. Physical examination revealed an ulcer on the right labia minora of 15 mm in diameter with fibrin. Patient was hospitalized for intravenous analgesia, progressed with a good response and was discharged after 4 days. At control a vulvar ulcer of 10 mm was noted on the right lip, with little fibrin and no pain.
Considering the patient’s clinic of recurrent aphthosis of non-infectious etiology and her family history, it is highly suggestive of a Behçet disease, so actually she is managed expectantly, emphasizing to look for eventual ophthalmological, cutaneous and neurological symptoms.

Comments: Despite most of genital ulcers having an infectious etiology, it is important to consider other conditions such as the inflammatory bowel disease or Behçet disease. Is not easy to distinguish them just by checking their appearance during an isolated episode, so we emphasize the importance of follow-up, family history and other complementary exams.