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Multisystem Langerhans cell histiocytosis in an adult non‐smoker treated with steroid therapy

We describe the case of a 29‐year‐old female non‐smoker who was treated with steroid therapy for a subacute exacerbation of multisystem Langerhans cell histiocytosis (MS‐LCH) with worsening lung, skin, and oral mucosal lesions. The patient developed pneumonia, and computed tomography (CT) showed mul...

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Autores principales: Ito, Haruka, Ito, Masaru, Kakuta, Yukio, Kaneko, Takeshi, Okudera, Koji, Ogura, Takashi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley & Sons, Ltd 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7290287/
https://www.ncbi.nlm.nih.gov/pubmed/32547768
http://dx.doi.org/10.1002/rcr2.603
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author Ito, Haruka
Ito, Masaru
Kakuta, Yukio
Kaneko, Takeshi
Okudera, Koji
Ogura, Takashi
author_facet Ito, Haruka
Ito, Masaru
Kakuta, Yukio
Kaneko, Takeshi
Okudera, Koji
Ogura, Takashi
author_sort Ito, Haruka
collection PubMed
description We describe the case of a 29‐year‐old female non‐smoker who was treated with steroid therapy for a subacute exacerbation of multisystem Langerhans cell histiocytosis (MS‐LCH) with worsening lung, skin, and oral mucosal lesions. The patient developed pneumonia, and computed tomography (CT) showed multiple thin‐walled cavities. Transbronchial lung cryobiopsy (TBLC) specimens revealed Langerhans cells, which were positive for CD1a and S‐100 expression. Similar histological findings were detected in the submandibular gland, skin, and tooth. On the basis of these findings, the patient was diagnosed with MS‐LCH and subsequently treated with steroid therapy. From the literature review, case reports of non‐smokers with pulmonary lesions that worsened and required treatment are rare. Almost all cases recurred and needed additional treatment. This case study contributes to our understanding of the potential role of steroid therapy in MS‐LCH treatment. Additionally, TBLC is a novel, potentially safer, diagnostic tool that has not been previously described for LCH.
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spelling pubmed-72902872020-06-15 Multisystem Langerhans cell histiocytosis in an adult non‐smoker treated with steroid therapy Ito, Haruka Ito, Masaru Kakuta, Yukio Kaneko, Takeshi Okudera, Koji Ogura, Takashi Respirol Case Rep Case Reports We describe the case of a 29‐year‐old female non‐smoker who was treated with steroid therapy for a subacute exacerbation of multisystem Langerhans cell histiocytosis (MS‐LCH) with worsening lung, skin, and oral mucosal lesions. The patient developed pneumonia, and computed tomography (CT) showed multiple thin‐walled cavities. Transbronchial lung cryobiopsy (TBLC) specimens revealed Langerhans cells, which were positive for CD1a and S‐100 expression. Similar histological findings were detected in the submandibular gland, skin, and tooth. On the basis of these findings, the patient was diagnosed with MS‐LCH and subsequently treated with steroid therapy. From the literature review, case reports of non‐smokers with pulmonary lesions that worsened and required treatment are rare. Almost all cases recurred and needed additional treatment. This case study contributes to our understanding of the potential role of steroid therapy in MS‐LCH treatment. Additionally, TBLC is a novel, potentially safer, diagnostic tool that has not been previously described for LCH. John Wiley & Sons, Ltd 2020-06-12 /pmc/articles/PMC7290287/ /pubmed/32547768 http://dx.doi.org/10.1002/rcr2.603 Text en © 2020 The Authors. Respirology Case Reports published by John Wiley & Sons Australia, Ltd on behalf of The Asian Pacific Society of Respirology This is an open access article under the terms of the http://creativecommons.org/licenses/by/4.0/ License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Reports
Ito, Haruka
Ito, Masaru
Kakuta, Yukio
Kaneko, Takeshi
Okudera, Koji
Ogura, Takashi
Multisystem Langerhans cell histiocytosis in an adult non‐smoker treated with steroid therapy
title Multisystem Langerhans cell histiocytosis in an adult non‐smoker treated with steroid therapy
title_full Multisystem Langerhans cell histiocytosis in an adult non‐smoker treated with steroid therapy
title_fullStr Multisystem Langerhans cell histiocytosis in an adult non‐smoker treated with steroid therapy
title_full_unstemmed Multisystem Langerhans cell histiocytosis in an adult non‐smoker treated with steroid therapy
title_short Multisystem Langerhans cell histiocytosis in an adult non‐smoker treated with steroid therapy
title_sort multisystem langerhans cell histiocytosis in an adult non‐smoker treated with steroid therapy
topic Case Reports
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7290287/
https://www.ncbi.nlm.nih.gov/pubmed/32547768
http://dx.doi.org/10.1002/rcr2.603
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