CASE REPORT: A LADY WITH RECURRENT PERIANAL ULCER
Nawshirwan Gafoor Rashid a
a Hiwa Hemato-Oncology Hospital-Slemani-KRI-Iraq, Kurdistan Board of Medical specialities, Slemani Hematology Center.
Submitted: 23 /2 /2022; Accepted: 15/5 /2020; Published: 21/6/2022
DOI Link: https://doi.org/10.17656/jsmc.10362
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.
Recurrent ulcer, Perianal ulcer.
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2. Kobayashi M, Tojo A. Langerhans cell histiocytosis in adults: Advances in pathophysiology and treatment. Cancer Sci. 2018; 109:3707–3713.
3. Axiotis CA, Merino MJ, Duray PH. Langerhans cell histiocytosis of the female genital tract. Cancer 1991; 67:1650–1660.
4. Campanati A, Simonetti O, Marconi B, Giuliodori K, Ganzetti G, Brandozzi G, Bernardini ML, Ranaldi R, Offidani A. Purely cutaneous Langerhans’ cell histiocytosis in an adult woman. Acta Derm Venereol. 2009; 89:299–301.
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