CASE REPORT: A LADY WITH RECURRENT PERIANAL ULCER
DOI:
https://doi.org/10.17656/jsmc.10362Keywords:
Recurrent ulcer, Perianal ulcerAbstract
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.
References
Wieland R, Flanagan J, Everett E, Mount S. Langerhans cell histiocytosis limited to the female genital tract: A literature review with three additional cases. Gynecol Oncol Rep 2017; 22:4‑8. DOI: https://doi.org/10.1016/j.gore.2017.08.005
Kobayashi M, Tojo A. Langerhans cell histiocytosis in adults: Advances in pathophysiology and treatment. Cancer Sci. 2018; 109:3707–3713. DOI: https://doi.org/10.1111/cas.13817
Axiotis CA, Merino MJ, Duray PH. Langerhans cell histiocytosis of the female genital tract. Cancer 1991; 67:1650–1660. DOI: https://doi.org/10.1002/1097-0142(19910315)67:6<1650::AID-CNCR2820670629>3.0.CO;2-T
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. DOI: https://doi.org/10.2340/00015555-0614
Downloads
Published
Issue
Section
License
Copyright (c) 2022 Nawshirwan Gafoor Rashid
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
This work is licensed under 