顔面軟部組織に発生したランゲルハンス細胞組織球症の1例

Langerhans cell histiocytosis (LCH) is a rare proliferative disorder characterized by the accumulation of Langerhans cells, and wide-ranging organ involvement. In the oral and maxillofacial region many cases have been reported in the hard tissue of jaws; cases occurring in facial soft tissue are rar...

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Published in日本口腔外科学会雑誌 Vol. 69; no. 6; pp. 298 - 303
Main Authors 伊原木, 聰一郎, 吉岡, 徳枝, 小野, 喜章, 長塚, 仁, 増井, 正典, 佐々木, 朗
Format Journal Article
LanguageJapanese
Published 公益社団法人 日本口腔外科学会 20.06.2023
Subjects
コルチコステロイド
ランゲルハンス細胞組織球症
局所注射
顔面軟部組織
Online AccessGet full text
ISSN0021-5163
2186-1579
DOI10.5794/jjoms.69.298

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Abstract Langerhans cell histiocytosis (LCH) is a rare proliferative disorder characterized by the accumulation of Langerhans cells, and wide-ranging organ involvement. In the oral and maxillofacial region many cases have been reported in the hard tissue of jaws; cases occurring in facial soft tissue are rare. We report a case of LCH arising in the facial soft tissue of a 26-year-old man who presented with an ulcerative lesion on the lower lip and erythema around the left eyelid skin.　Tissue biopsies of both the lower lip and left eyelid skin lesions were performed, and a histological diagnosis of LCH was obtained for both lesions. The lesion on the lower lip was treated by corticosteroid injection, and the lesion around the left eyelid skin was treated by corticosteroid ointment. One month later, the lesion on the lower lip had shrunk, and the lesion around the left eyelid was improving. The patient was followed up for eight years after the treatment, and there was no recurrence or appearance of lesions in any other organs. Although skin-mucosal limited LCH usually indicates an indolent clinical course, there is a secondary risk of developing hematopoietic tumors and long-term follow-up is required.
AbstractList Langerhans cell histiocytosis (LCH) is a rare proliferative disorder characterized by the accumulation of Langerhans cells, and wide-ranging organ involvement. In the oral and maxillofacial region many cases have been reported in the hard tissue of jaws; cases occurring in facial soft tissue are rare. We report a case of LCH arising in the facial soft tissue of a 26-year-old man who presented with an ulcerative lesion on the lower lip and erythema around the left eyelid skin.　Tissue biopsies of both the lower lip and left eyelid skin lesions were performed, and a histological diagnosis of LCH was obtained for both lesions. The lesion on the lower lip was treated by corticosteroid injection, and the lesion around the left eyelid skin was treated by corticosteroid ointment. One month later, the lesion on the lower lip had shrunk, and the lesion around the left eyelid was improving. The patient was followed up for eight years after the treatment, and there was no recurrence or appearance of lesions in any other organs. Although skin-mucosal limited LCH usually indicates an indolent clinical course, there is a secondary risk of developing hematopoietic tumors and long-term follow-up is required.
Author 佐々木, 朗
吉岡, 徳枝
伊原木, 聰一郎
増井, 正典
小野, 喜章
長塚, 仁
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References 18) Ishii R, Morimoto A, et al : High serum values of soluble CD154, IL-2 receptor, RANKL and osteoprotegerin in Langerhans cell histiocytosis．Pediatr Blood Cancer 47: 194-199, 2006.
16) Kilic E, Er N, et al : Oral mucosal involvement in Langerhans’ cell histiocytosis: long-term follow-up of a rare case．Aust Dent J 56: 433-436, 2011.
15) Milián MA, Bagán JV, et al : Langerhans' cell histiocytosis restricted to the oral mucosa．Oral Surg Oral Med Oral Pathol Oral Radiol Endod 91: 76-79, 2001.
24) Weitzman S and Egeler RM : Langerhans cell histiocytosis: update for the pediatrician．Curr Opin Pediatr 20: 23-29, 2008.
10)Val-Bernal JF, González-Vela MC, et al : Localized eosinophilic (Langerhans’cell) granuloma of the lower lip. A lesion that may cause diagnostic error．J Cutan Pathol 36: 1109-1113, 2009.
11) Yashoda V, Reddy ESK, et al : Solitary extragnathic langerhans cell histiocytosis - a rare case．J Clin Diagn Res 9: Zd22-23, 2015.
2)Götz G and Fichter J : Langerhans’-cell histiocytosis in 58 adults．Eur J Med Res 9: 510-514, 2004.
25) Edelbroek JR, Vermeer MH, et al : Langerhans cell histiocytosis first presenting in the skin in adults: frequent association with a second haematological malignancy．Br J Dermatol 167: 1287-1294, 2012.
27) Caux C, Dezutter-Dambuyant C, et al : GM-CSF and TNF-alpha cooperate in the generation of dendritic Langerhans cells．Nature 360: 258-261, 1992.
21) Albano D, Bosio G, et al : Role of (18) F-FDG PET/ CT in patients affected by Langerhans cell histiocytosis．Jpn J Radiol 35: 574-583, 2017.
5) Carstensen H and Ornvold K : The epidemiology of LCH in children in Denmark．Med Pediatr Oncol 21: 387-388, 1993.
23) 森本　哲：組織球症の病態解明と治療の進歩．日小児血がん会誌 53: 428-435, 2017.
20) Kumar R and Balachandran S : Relative roles of radionuclide scanning and radiographic imaging in eosinophilic granuloma．Clin Nucl Med 5: 538-542, 1980.
12) Hashimoto K, Takahashi S, et al : Eosinophilic Granuloma: Presence of OKT6-Positive Cells and Good Response to Intralesional Steroid．Archives of Dermatology 121: 770-774, 1985.
22) 厚生労働科学難治性疾患克服研究事業 乳児ランゲルハンス細胞組織球症の病態解明と診療研究班： LCH 病理診断ガイドライン(Ver.2010). Available at: http://www.jlsg.jp/korosho_kenkyuhan/dl_files/guideline_byori.pdf. Accessed April 12, 2022.
3)Aricò M, Girschikofsky M, et al : Langerhans cell histiocytosis in adults. Report from the International Registry of the Histiocyte Society．Eur J Cancer 39: 2341-2348, 2003.
9) Iwasaki T, Takahashi I, et al : Cutaneous Langerhans cell histiocytosis in elderly with chronic myelomonocytic leukemia．J Dermatol 41: 262-265, 2014.
19) Rosso DA, Roy A, et al : Prognostic value of soluble interleukin 2 receptor levels in Langerhans cell histiocytosis．Br J Haematol 117: 54-58, 2002.
1) Favara BE, Feller AC, et al : Contemporary classification of histiocytic disorders. The WHO Committee On Histiocytic/Reticulum Cell Proliferations. Reclassification Working Group of the Histiocyte Society．Med Pediatr Oncol 29: 157-166, 1997.
13) Bottomley WK, Gabriel SA, et al : Histiocytosis X: Report of an oral soft tissue lesion without bony involvement．Oral Surgery, Oral Medicine, Oral Pathology 63: 228-231, 1987.
14) Cleveland DB, Goldberg KM, et al : Langerhans’ cell histiocytosis: report of three cases with unusual oral soft tissue involvement．Oral Surg Oral Med Oral Pathol Oral Radiol Endod 82: 541-548, 1996.
26) McClain KL and Kozinetz CA : A phase II trial using thalidomide for Langerhans cell histiocytosis．Pediatr Blood Cancer 48: 44-49, 2007.
6) Maia RC, de Rezende LM, et al : Langerhans cell histiocytosis: differences and similarities in long-term outcome of paediatric and adult patients at a single institutional centre．Hematology 20: 83-92, 2015.
4) Maia RC, de Rezende LM, et al : Langerhans cell histiocytosis: differences and similarities in long-term outcome of paediatric and adult patients at a single institutional centre．Hematology 20: 83-92, 2015.
28) 中谷倫子， 成川　玄， 他：コルチコステロイドの局所注射が奏効した下顎骨ランゲルハンス細胞組織球症の1例．日口外誌 57: 128-132, 2011.
7) Hartman KS : Histiocytosis X: a review of 114 cases with oral involvement．Oral Surg Oral Med Oral Pathol 49: 38-54, 1980.
29) Haupt R, Nanduri V, et al : Permanent consequences in Langerhans cell histiocytosis patients: a pilot study from the Histiocyte Society-Late Effects Study Group．Pediatr Blood Cancer 42: 438-444, 2004.
17) Luder CM, Nordmann TM, et al : Histiocytosis - cutaneous manifestations of hematopoietic neoplasm and non-neoplastic histiocytic proliferations．J Eur Acad Dermatol Venereol 32: 926-934, 2018.
8) Hicks J and Flaitz CM : Langerhans cell histiocytosis: current insights in a molecular age with emphasis on clinical oral and maxillofacial pathology practice．Oral Surg Oral Med Oral Pathol Oral Radiol Endod 100: S42-66, 2005.
References_xml – reference: 20) Kumar R and Balachandran S : Relative roles of radionuclide scanning and radiographic imaging in eosinophilic granuloma．Clin Nucl Med 5: 538-542, 1980.
– reference: 17) Luder CM, Nordmann TM, et al : Histiocytosis - cutaneous manifestations of hematopoietic neoplasm and non-neoplastic histiocytic proliferations．J Eur Acad Dermatol Venereol 32: 926-934, 2018.
– reference: 22) 厚生労働科学難治性疾患克服研究事業 乳児ランゲルハンス細胞組織球症の病態解明と診療研究班： LCH 病理診断ガイドライン(Ver.2010). Available at: http://www.jlsg.jp/korosho_kenkyuhan/dl_files/guideline_byori.pdf. Accessed April 12, 2022.
– reference: 1) Favara BE, Feller AC, et al : Contemporary classification of histiocytic disorders. The WHO Committee On Histiocytic/Reticulum Cell Proliferations. Reclassification Working Group of the Histiocyte Society．Med Pediatr Oncol 29: 157-166, 1997.
– reference: 9) Iwasaki T, Takahashi I, et al : Cutaneous Langerhans cell histiocytosis in elderly with chronic myelomonocytic leukemia．J Dermatol 41: 262-265, 2014.
– reference: 8) Hicks J and Flaitz CM : Langerhans cell histiocytosis: current insights in a molecular age with emphasis on clinical oral and maxillofacial pathology practice．Oral Surg Oral Med Oral Pathol Oral Radiol Endod 100: S42-66, 2005.
– reference: 27) Caux C, Dezutter-Dambuyant C, et al : GM-CSF and TNF-alpha cooperate in the generation of dendritic Langerhans cells．Nature 360: 258-261, 1992.
– reference: 25) Edelbroek JR, Vermeer MH, et al : Langerhans cell histiocytosis first presenting in the skin in adults: frequent association with a second haematological malignancy．Br J Dermatol 167: 1287-1294, 2012.
– reference: 15) Milián MA, Bagán JV, et al : Langerhans' cell histiocytosis restricted to the oral mucosa．Oral Surg Oral Med Oral Pathol Oral Radiol Endod 91: 76-79, 2001.
– reference: 2)Götz G and Fichter J : Langerhans’-cell histiocytosis in 58 adults．Eur J Med Res 9: 510-514, 2004.
– reference: 14) Cleveland DB, Goldberg KM, et al : Langerhans’ cell histiocytosis: report of three cases with unusual oral soft tissue involvement．Oral Surg Oral Med Oral Pathol Oral Radiol Endod 82: 541-548, 1996.
– reference: 6) Maia RC, de Rezende LM, et al : Langerhans cell histiocytosis: differences and similarities in long-term outcome of paediatric and adult patients at a single institutional centre．Hematology 20: 83-92, 2015.
– reference: 19) Rosso DA, Roy A, et al : Prognostic value of soluble interleukin 2 receptor levels in Langerhans cell histiocytosis．Br J Haematol 117: 54-58, 2002.
– reference: 5) Carstensen H and Ornvold K : The epidemiology of LCH in children in Denmark．Med Pediatr Oncol 21: 387-388, 1993.
– reference: 21) Albano D, Bosio G, et al : Role of (18) F-FDG PET/ CT in patients affected by Langerhans cell histiocytosis．Jpn J Radiol 35: 574-583, 2017.
– reference: 3)Aricò M, Girschikofsky M, et al : Langerhans cell histiocytosis in adults. Report from the International Registry of the Histiocyte Society．Eur J Cancer 39: 2341-2348, 2003.
– reference: 26) McClain KL and Kozinetz CA : A phase II trial using thalidomide for Langerhans cell histiocytosis．Pediatr Blood Cancer 48: 44-49, 2007.
– reference: 16) Kilic E, Er N, et al : Oral mucosal involvement in Langerhans’ cell histiocytosis: long-term follow-up of a rare case．Aust Dent J 56: 433-436, 2011.
– reference: 24) Weitzman S and Egeler RM : Langerhans cell histiocytosis: update for the pediatrician．Curr Opin Pediatr 20: 23-29, 2008.
– reference: 28) 中谷倫子， 成川　玄， 他：コルチコステロイドの局所注射が奏効した下顎骨ランゲルハンス細胞組織球症の1例．日口外誌 57: 128-132, 2011.
– reference: 29) Haupt R, Nanduri V, et al : Permanent consequences in Langerhans cell histiocytosis patients: a pilot study from the Histiocyte Society-Late Effects Study Group．Pediatr Blood Cancer 42: 438-444, 2004.
– reference: 12) Hashimoto K, Takahashi S, et al : Eosinophilic Granuloma: Presence of OKT6-Positive Cells and Good Response to Intralesional Steroid．Archives of Dermatology 121: 770-774, 1985.
– reference: 13) Bottomley WK, Gabriel SA, et al : Histiocytosis X: Report of an oral soft tissue lesion without bony involvement．Oral Surgery, Oral Medicine, Oral Pathology 63: 228-231, 1987.
– reference: 10)Val-Bernal JF, González-Vela MC, et al : Localized eosinophilic (Langerhans’cell) granuloma of the lower lip. A lesion that may cause diagnostic error．J Cutan Pathol 36: 1109-1113, 2009.
– reference: 4) Maia RC, de Rezende LM, et al : Langerhans cell histiocytosis: differences and similarities in long-term outcome of paediatric and adult patients at a single institutional centre．Hematology 20: 83-92, 2015.
– reference: 11) Yashoda V, Reddy ESK, et al : Solitary extragnathic langerhans cell histiocytosis - a rare case．J Clin Diagn Res 9: Zd22-23, 2015.
– reference: 7) Hartman KS : Histiocytosis X: a review of 114 cases with oral involvement．Oral Surg Oral Med Oral Pathol 49: 38-54, 1980.
– reference: 18) Ishii R, Morimoto A, et al : High serum values of soluble CD154, IL-2 receptor, RANKL and osteoprotegerin in Langerhans cell histiocytosis．Pediatr Blood Cancer 47: 194-199, 2006.
– reference: 23) 森本　哲：組織球症の病態解明と治療の進歩．日小児血がん会誌 53: 428-435, 2017.
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SubjectTerms コルチコステロイド
ランゲルハンス細胞組織球症
局所注射
顔面軟部組織
Title 顔面軟部組織に発生したランゲルハンス細胞組織球症の1例
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