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Isolated Cutaneous Langerhans Cell Histiocytosis Presenting in an Adult Male

Langerhans cell histiocytosis (LCH) is an infrequent clonal proliferative disorder of myeloid dendritic cells. It has a wide variety of cutaneous manifestations and retains the possibility of systemic implications. Because LCH is predominantly a disease of childhood, there are well-established clini...

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Autores principales: Fronek, Lisa F, Grubbs, Hailey, Dorton, David W, Miller, Richard
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7500710/
https://www.ncbi.nlm.nih.gov/pubmed/32963902
http://dx.doi.org/10.7759/cureus.9861
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author Fronek, Lisa F
Grubbs, Hailey
Dorton, David W
Miller, Richard
author_facet Fronek, Lisa F
Grubbs, Hailey
Dorton, David W
Miller, Richard
author_sort Fronek, Lisa F
collection PubMed
description Langerhans cell histiocytosis (LCH) is an infrequent clonal proliferative disorder of myeloid dendritic cells. It has a wide variety of cutaneous manifestations and retains the possibility of systemic implications. Because LCH is predominantly a disease of childhood, there are well-established clinical definitions, as well as guidelines regarding workup and treatment, in the context of pediatric disease. Here we present a case of isolated cutaneous LCH in an adult male, followed by a discussion of our diagnostic plan and treatment course. The patient exhibited a small, excoriated, yellow papule on his inferior forehead during a skin examination. The specimen underwent tangential shave biopsy; histopathologic evaluation with appropriate immunohistochemical staining confirmed a diagnosis of cutaneous LCH. After thorough investigation via serologic and imaging diagnostics, we confirmed isolated cutaneous disease. The patient underwent wide local excision (WLE) with no evidence of recurrence. It is crucial to appropriately screen all patients diagnosed with cutaneous LCH for internal organ involvement. The authors aim to highlight the need for further investigations to ultimately dictate standardized management and treatment for isolated cutaneous LCH in the adult population.
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spelling pubmed-75007102020-09-21 Isolated Cutaneous Langerhans Cell Histiocytosis Presenting in an Adult Male Fronek, Lisa F Grubbs, Hailey Dorton, David W Miller, Richard Cureus Dermatology Langerhans cell histiocytosis (LCH) is an infrequent clonal proliferative disorder of myeloid dendritic cells. It has a wide variety of cutaneous manifestations and retains the possibility of systemic implications. Because LCH is predominantly a disease of childhood, there are well-established clinical definitions, as well as guidelines regarding workup and treatment, in the context of pediatric disease. Here we present a case of isolated cutaneous LCH in an adult male, followed by a discussion of our diagnostic plan and treatment course. The patient exhibited a small, excoriated, yellow papule on his inferior forehead during a skin examination. The specimen underwent tangential shave biopsy; histopathologic evaluation with appropriate immunohistochemical staining confirmed a diagnosis of cutaneous LCH. After thorough investigation via serologic and imaging diagnostics, we confirmed isolated cutaneous disease. The patient underwent wide local excision (WLE) with no evidence of recurrence. It is crucial to appropriately screen all patients diagnosed with cutaneous LCH for internal organ involvement. The authors aim to highlight the need for further investigations to ultimately dictate standardized management and treatment for isolated cutaneous LCH in the adult population. Cureus 2020-08-19 /pmc/articles/PMC7500710/ /pubmed/32963902 http://dx.doi.org/10.7759/cureus.9861 Text en Copyright © 2020, Fronek et al. http://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Dermatology
Fronek, Lisa F
Grubbs, Hailey
Dorton, David W
Miller, Richard
Isolated Cutaneous Langerhans Cell Histiocytosis Presenting in an Adult Male
title Isolated Cutaneous Langerhans Cell Histiocytosis Presenting in an Adult Male
title_full Isolated Cutaneous Langerhans Cell Histiocytosis Presenting in an Adult Male
title_fullStr Isolated Cutaneous Langerhans Cell Histiocytosis Presenting in an Adult Male
title_full_unstemmed Isolated Cutaneous Langerhans Cell Histiocytosis Presenting in an Adult Male
title_short Isolated Cutaneous Langerhans Cell Histiocytosis Presenting in an Adult Male
title_sort isolated cutaneous langerhans cell histiocytosis presenting in an adult male
topic Dermatology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7500710/
https://www.ncbi.nlm.nih.gov/pubmed/32963902
http://dx.doi.org/10.7759/cureus.9861
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