Volume 12 ,Issue 2 ,June 2022 ,Pages 207-210
1 a Hiwa Hemato-Oncology Hospital-Slemani-KRI-Iraq, Kurdistan Board of Medical specialities, Slemani Hematology Center.
INTRODUCTION
Langerhans cell histiocytosis (LCH) is a rare systemic disorder characterized by clonal proliferation of
bone‑marrow-derived Langerhans cells in various systems. Lesions on the genital tract are uncommon, with
only a few reports of isolated vulvar LCH (1). It is characterized by accumulating CD1a+/Langerin+ LCH
cells and wide-ranging organ involvement (2). The presentation can vary from single organ involvement to
disseminated, multi-system disease. Clinical aggressiveness is equally variable. In 1939 Andrews first described
LCH of the female reproductive tract. Subsequently, four patterns of involvement have been identified: (a) pure
genital LCH, (b) genital tract LCH with subsequent multi-organ involvement, (c) oral or cutaneous LCH with
subsequent genital and multi-organ involvement, and (d) diabetes insipidus with organ involvement (3).here
our case at the beginning she had only perianal skin involvement which was completely resected but after
two years she relapsed with perianal & valvar ulcer, again totally resected and the result of histopathology &
immunohistochemistry with positive CD1a, CD 207 & S100 confirms recurrence of the same pathology