Behçet’s disease in an adolescent patient

Behçet’s disease (BD) is a chronic and multisystematic inflammatory disorder characterized by oral and genital mucous “aphthous-like” recurrent ulcers, uveitis, and skin lesions. Oral ulcerations are present in more than 90% of patients being often the first clinical manifestation. Although the actual etiology is still unclear, BD symptoms are considered to be based on the correlation between the genetic factors (HLA B51) and the triggering extrinsic factors (i.e. oral unhygienic condition). Pediatric onset BD is an uncommon disease that presents before the age of 16 years and has a distinct clinical presentation of recurrent abdominal pain and episodes of fever. We report an interesting case of a 14 years old Caucasian male referring to our Unit for 3-years history of recurrent oral lesions. From a systemic point of view, the patient reported a deficiency of glucose-6-phosphate dehydrogenase, abdominal pain and meteorism. The intra-oral examination revealed “aphthous-like” ulcers on the tip of the tongue and lower labial mucosa and scarring areas in the lower labial mucosa. Furthermore, the lower lip appears swollen and with an increased consistency. Due to the age of the patient and the clinical signs and symptoms we consider as potential diagnosis a gastrointestinal disease as coeliac disease and Chron disease. However, serological screening for celiac disease was negative as well as faecal calprotectin; while, deficiencies of iron and folate and increased VES were showed. After 1 month the patient presented with a genital “aphthous-like” ulcer. So a BD was suspected and the research of HLA B51 was positive. The patient was referred to the ophthalmologist and dermatologist who excluded eyes and skin involvement. This case underlines that the presence of complex oral aphthous episodes also in paediatric patients should prompt the clinician to suspect the presence of an underlying systemic disease, crucial step for its early diagnosis and treatment.