Adult-onset perianal Langerhans cell histiocytosis presenting as pruritus ani: a case report and review of the literature

Abstract Background Langerhans cells belong to the histiocytic system and give rise to two tumors: Langerhans cell histiocytosis and Langerhans cell sarcoma. Clinical aggressiveness and degree of atypia distinguish the two neoplasms. Langerhans cell histiocytosis can infiltrate a single or multiple...

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Main Authors: Marah Hamdan, Jesse C. Qiao, Vid Fikfak
Format: Article
Language:English
Published: BMC 2021-07-01
Series:Journal of Medical Case Reports
Subjects:
Online Access:https://doi.org/10.1186/s13256-021-02924-0
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author Marah Hamdan
Jesse C. Qiao
Vid Fikfak
author_facet Marah Hamdan
Jesse C. Qiao
Vid Fikfak
author_sort Marah Hamdan
collection DOAJ
description Abstract Background Langerhans cells belong to the histiocytic system and give rise to two tumors: Langerhans cell histiocytosis and Langerhans cell sarcoma. Clinical aggressiveness and degree of atypia distinguish the two neoplasms. Langerhans cell histiocytosis can infiltrate a single or multiple organ systems and particularly affects bone, skin, and lymph nodes. Perianal cutaneous Langerhans cell histiocytosis is a rare condition in adults, with 15 cases reported in the literature. Case We present the case of a 50-year-old hispanic man who presented with a 9-month history of pruritus ani and a personal history of diabetes insipidus. Punch biopsy confirmed a lesion of Langerhans cells origin but could not exclude Langerhans cell sarcoma because of limited sample size. An additional biopsy was planned as well as a positron emission tomography scan to determine the extent of disease spread. While the patient failed to follow up for repeat biopsy, the positron emission tomography scan was performed and was negative for metastatic disease. A stable perianal lesion of Langerhans cell histiocytosis with benign clinical features in a 50-year-old male despite lack of treatment is extremely rare and has not been described in the literature so far. Here, we review the presentation and workup of patients with Langerhans cell histiocytosis, review the relevant literature, and discuss treatment planning. Conclusion Perianal Langerhans cell histiocytosis is rare, and there should be a high index of suspicion with chronic or new perianal lesions, especially in a patient with a history of diabetes insipidus. It is also important to consider the patient’s full clinical course when it is not possible to reach a definitive pathological diagnosis before management.
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spelling doaj.art-c101cdb66e004d56a863f429b916276f2022-12-21T19:57:52ZengBMCJournal of Medical Case Reports1752-19472021-07-0115111210.1186/s13256-021-02924-0Adult-onset perianal Langerhans cell histiocytosis presenting as pruritus ani: a case report and review of the literatureMarah Hamdan0Jesse C. Qiao1Vid Fikfak2Departments of Surgery, Texas Tech University Health Sciences CenterPathology at Texas Tech University Health Sciences CenterDepartments of Surgery, Texas Tech University Health Sciences CenterAbstract Background Langerhans cells belong to the histiocytic system and give rise to two tumors: Langerhans cell histiocytosis and Langerhans cell sarcoma. Clinical aggressiveness and degree of atypia distinguish the two neoplasms. Langerhans cell histiocytosis can infiltrate a single or multiple organ systems and particularly affects bone, skin, and lymph nodes. Perianal cutaneous Langerhans cell histiocytosis is a rare condition in adults, with 15 cases reported in the literature. Case We present the case of a 50-year-old hispanic man who presented with a 9-month history of pruritus ani and a personal history of diabetes insipidus. Punch biopsy confirmed a lesion of Langerhans cells origin but could not exclude Langerhans cell sarcoma because of limited sample size. An additional biopsy was planned as well as a positron emission tomography scan to determine the extent of disease spread. While the patient failed to follow up for repeat biopsy, the positron emission tomography scan was performed and was negative for metastatic disease. A stable perianal lesion of Langerhans cell histiocytosis with benign clinical features in a 50-year-old male despite lack of treatment is extremely rare and has not been described in the literature so far. Here, we review the presentation and workup of patients with Langerhans cell histiocytosis, review the relevant literature, and discuss treatment planning. Conclusion Perianal Langerhans cell histiocytosis is rare, and there should be a high index of suspicion with chronic or new perianal lesions, especially in a patient with a history of diabetes insipidus. It is also important to consider the patient’s full clinical course when it is not possible to reach a definitive pathological diagnosis before management.https://doi.org/10.1186/s13256-021-02924-0Langerhans cell histiocytosisLangerhans cell sarcomaPerianal Langerhans histiocytosisCase report
spellingShingle Marah Hamdan
Jesse C. Qiao
Vid Fikfak
Adult-onset perianal Langerhans cell histiocytosis presenting as pruritus ani: a case report and review of the literature
Journal of Medical Case Reports
Langerhans cell histiocytosis
Langerhans cell sarcoma
Perianal Langerhans histiocytosis
Case report
title Adult-onset perianal Langerhans cell histiocytosis presenting as pruritus ani: a case report and review of the literature
title_full Adult-onset perianal Langerhans cell histiocytosis presenting as pruritus ani: a case report and review of the literature
title_fullStr Adult-onset perianal Langerhans cell histiocytosis presenting as pruritus ani: a case report and review of the literature
title_full_unstemmed Adult-onset perianal Langerhans cell histiocytosis presenting as pruritus ani: a case report and review of the literature
title_short Adult-onset perianal Langerhans cell histiocytosis presenting as pruritus ani: a case report and review of the literature
title_sort adult onset perianal langerhans cell histiocytosis presenting as pruritus ani a case report and review of the literature
topic Langerhans cell histiocytosis
Langerhans cell sarcoma
Perianal Langerhans histiocytosis
Case report
url https://doi.org/10.1186/s13256-021-02924-0
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AT jessecqiao adultonsetperianallangerhanscellhistiocytosispresentingaspruritusaniacasereportandreviewoftheliterature
AT vidfikfak adultonsetperianallangerhanscellhistiocytosispresentingaspruritusaniacasereportandreviewoftheliterature