Acute Vulvar Ulceration as the Main Presenting Manifestation of Hemophagocytic Syndrome

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...

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Veröffentlicht in:Pediatric hematology and oncology 2003, Vol.20 (5), p.421-425
Hauptverfasser: Kobayashi, Ken-ichiro, Usami, Ikuya, Kubota, Masaru, Nishio, Toshikazu
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Sprache:eng
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Zusammenfassung: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 &#103 -2-microglobulin ( &#103 -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 &#103 -2M was the most sensitive marker for the evaluation of the disease activity of HPS.
ISSN:0888-0018
1521-0669
DOI:10.1080/08880010390203990