Oro-genital ulcers with a positive pathergy test in acute myeloid leukaemia.

An 18-year old unmarried girl presented with intermittent high grade fever and painful oro-genital ulcers of one month duration. Her past medical history, sexual history and family history were unremarkable. Clinically she was ill with tender mucosal ulcers in the mouth as well as in the genitals. There was no lymphadenopathy, hepatosplenomegaly or a skin rash. Her eyes were normal. One week later, she developed an asymmetrical, large joint arthritis involving the knees and ankles and painful tender red nodules over the shins, suggestive of erythema nodosum. Within 48 hours of insertion of intravenous cannulae as well as following venepuncture, pustular vesicles appeared at puncture sites (a positive pathergy test). A clinical diagnosis of Behçet’s disease was made.