title: Acute vulvar ulceration as the main presenting manifestation of hemophagocytic syndrome.
abstract: A 14-year-old female exhibited an acute vulvar ulcer during the course of hemophagocytic syndrome (HPS). The patient presented persistent high fever and a deep painful vulvar ulcer lasting for more than 2 weeks. Neither infection with sexually transmitted agents nor autoimmune disorder were found to be positive. The presence of hemophagocytosis in the bone marrow and elevation of urinary beta-2-microglobulin (beta-2M) prompted the diagnosis of HPS. Acute vulvar ulcer is rare, but it should be recognized as a mucous manifestation of HPS. During the clinical course, urinary beta-2M was the most sensitive marker for the evaluation of the disease activity of HPS.
authors.affiliation: Department of Pediatrics, Kobe City General Hospital, Kobe, Japan. kenichiro_kobayashi@medical.general.hp.city.kobe.jp
authors.forename: Ken-ichiro
authors.initials: K
authors.lastname: Kobayashi
authors.forename: Ikuya
authors.initials: I
authors.lastname: Usami
authors.forename: Masaru
authors.initials: M
authors.lastname: Kubota
authors.forename: Toshikazu
authors.initials: T
authors.lastname: Nishio
mesh_terms: D000208:Acute Disease; D000293:Adolescent; D015415:Biomarkers / Q000652:urine; D001856:Bone Marrow Examination; D005260:Female; D015616:Histiocytosis, Non-Langerhans-Cell / Q000150:complications* / Q000175:diagnosis* / Q000652:urine; D006801:Humans; D014456:Ulcer / Q000209:etiology*; D014845:Vulvar Diseases / Q000209:etiology*; D001613:beta 2-Microglobulin / Q000652:urine*
chemical_list: D015415:Biomarkers; D001613:beta 2-Microglobulin