Case Report
Copyright ©The Author(s) 2023. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Dec 6, 2023; 11(34): 8164-8169
Published online Dec 6, 2023. doi: 10.12998/wjcc.v11.i34.8164
Adult localized Langerhans cell histiocytosis: A case report
Pan-Pan Yang, Su-Ye Hu, Xu-Ya Chai, Xiao-Meng Shi, Li-Xia Liu, Ling-E Li
Pan-Pan Yang, Su-Ye Hu, Xu-Ya Chai, Xiao-Meng Shi, Ling-E Li, Department of Dermatology, Shijiazhuang TCM Hospital, Shijiazhuang 050051, Hebei Province, China
Li-Xia Liu, Department of Pathology, Shijiazhuang TCM Hospital, Shijiazhuang 050051, Hebei Province, China
Co-first authors: Pan-Pan Yang and Su-Ye Hu.
Author contributions: Chai XY and Yang PP performed laboratory testing and clinical data collection; Shi XM and Liu LX performed pathological studies; Yang PP and Hu SY drafted the manuscript; Hu SY and Li LE critically revised the manuscript for important intellectual content; all authors have read and approved the final version. Yang PP and Hu SY contributed equally to this work as co-first authors. The reasons for designating Yang PP and Hu SY as co-first authors are threefold. First, the research was performed as a collaborative effort, and the designation of co-first authorship accurately reflects the distribution of responsibilities and burdens associated with the time and effort required to complete the study and the resultant paper. This also ensures effective communication and management of post-submission matters, ultimately enhancing the paper's quality and reliability. Second, the overall research team encompassed authors with a variety of expertise and skills from different fields, and the designation of co-first authors best reflect this diversity. This also promotes the most comprehensive and in-depth examination of the research topic, ultimately enriching readers' understanding by offering various expert perspectives. Third, Yang PP and Hu SY contributed efforts of equal substance throughout the research process. The choice of these researchers as co-first authors acknowledges and respects this equal contribution, while recognizing the spirit of teamwork and collaboration of this study. In summary, we believe that designating Yang PP and Hu SY as co-first authors is fitting for our manuscript as it accurately reflects our team's collaborative spirit, equal contributions, and diversity.
Supported by Traditional Chinese Medicine Research Program of Hebei Provincial Administration of Traditional Chinese Medicine, No. 2022465.
Informed consent statement: Informed written consent was obtained from the patient for the publication of this case report.
Conflict-of-interest statement: The authors declare that they have no conflict of interest to disclose.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Ling-E Li, BSc, Chief Physician, Department of Dermatology, Shijiazhuang TCM Hospital, No. 233 Zhongshan West Road, Qiaoxi District, Shijiazhuang 050051, Hebei Province, China. zyypfk2008@126.com
Received: September 1, 2023
Peer-review started: September 1, 2023
First decision: October 17, 2023
Revised: October 30, 2023
Accepted: November 27, 2023
Article in press: November 27, 2023
Published online: December 6, 2023
Processing time: 96 Days and 7.4 Hours
Abstract
BACKGROUND

Langerhans cell histiocytosis (LCH) is a rare clonal proliferative disease of Langerhans cells with unknown pathogenesis. An increasing number of clinicians recognize that LCH has a wide clinical spectrum and a highly varied course. Adults rarely develop LCH. Here, we report a case of adult localized LCH.

CASE SUMMARY

A 32-year-old woman presented with plaques and ulcers on the vulva and crissum, accompanied by pain that persisted for more than one year. Physical examination revealed a red-infiltrating plaque with ulcerations and exudates in the vulva and crissum. Pathological examination revealed a diffuse infiltration of lymphocytes, eosinophilic granulocytes, and histiocytoid cells in the superficial dermis. Proliferative histiocytoid cells showed mild atypia, partly with kidney-shaped nuclei. Immunohistochemical examination showed that the histiocytoid cells were positive for S100 protein and CD1 and weakly positive for CD68 (20% +), with a Ki-67 index of 30%. Laboratory tests did not reveal any other systemic damage. The patient was diagnosed with adult localized LCH and was prescribed oral prednisone (20 mg) once daily. The skin lesions gradually improved and are still being followed-up.

CONCLUSION

Adult localized LCH is rare and must be differentiated from other common conditions.

Keywords: Langerhans cell histiocytosis; Adult; Vulva; Crissum; S100; Case report

Core Tip: Langerhans cell histiocytosis (LCH) is a rare clonal proliferative disease of Langerhans cells that express an immunophenotype positive for S100 protein, CD1 (CD1a), and Langerin (CD207) and contain cytoplasmic Birbeck granules. Adult localized LCH presenting with plaques and ulcers on the vulva and crissum is rare and must be differentiated from other common conditions.