{"title":"老年男性患者多中心网状组织细胞增多症的罕见病例","authors":"Rafael Everton Assunção Ribeiro da Costa","doi":"10.30733/std.2023.01716","DOIUrl":null,"url":null,"abstract":"Multicentric reticulohistiocytosis (MRH) is a rare systemic proliferative granulomatous disease of non-Langerhans cells. The aim of this study is \nto report a case of multicentric reticulohistiocytosis that is easily misdiagnosed in clinical practice. A 74-year-old man sought the Rheumatology \noffice in December 2022 with a main complaint of arthralgia for six months, associated with morning stiffness, joint swelling, and upper and lower \nlimb weakness. On physical examination, papules on the dorsal surface of the proximal interphalangeal joints, base of the nose, and lateral surface \nof the right hallux were observed. Hyperemia was also identified on the back, and edema of the proximal and distal interphalangeal joints, wrists \nand knees. Laboratory tests revealed ESR and ANA within normal limits. Tests were negative for Anti-Sm, Anti-RO, rheumatoid factor, Anti-CCP, \nHIV, and HCV. Chest CT-scan showed right axillary lymph node enlargement, measuring 2.7 cm, atheromatous aorta, and streaks with scattered \natelectasis in the lower lobe, without other changes. On total abdominal ultrasound, prostate enlargement was detected. Immunohistochemistry \nof skin biopsy identified atypical acinar proliferation. Histopathology skin biopsy stained with hematoxylin-eosin (January 2023) showed a \ndense histiocytic infiltrate. Detail of the histiocytic component displayed “ground glass” eosinophilic cytoplasm. Immunohistochemistry was \nnegative for S100 and CD34 and positive for CD68. Final diagnosis was MRH. The patient underwent treatment with drugs. In conclusion, clinical \nmanifestations, including skin and joint changes, with signs and symptoms of rheumatic disorders (e.g. dermatomyositis and rheumatoid arthritis) \nsimilar to those of MRH are expected in rheumatology practice. However, knowledge about disease progression in MRH is limited. Therefore, this \ncase report is described due to its rarity \nKeywords: Non-Langerhans-Cell Histiocytosis, Pathology, Muticentric Reticulohistiocytosis, Rheumatological Diseases,","PeriodicalId":332557,"journal":{"name":"Selcuk Tip Dergisi","volume":null,"pages":null},"PeriodicalIF":0.0000,"publicationDate":"2024-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"A Rare Case of Multicentric Reticulohistiocytosis in An Elderly Male Patient\",\"authors\":\"Rafael Everton Assunção Ribeiro da Costa\",\"doi\":\"10.30733/std.2023.01716\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Multicentric reticulohistiocytosis (MRH) is a rare systemic proliferative granulomatous disease of non-Langerhans cells. The aim of this study is \\nto report a case of multicentric reticulohistiocytosis that is easily misdiagnosed in clinical practice. A 74-year-old man sought the Rheumatology \\noffice in December 2022 with a main complaint of arthralgia for six months, associated with morning stiffness, joint swelling, and upper and lower \\nlimb weakness. On physical examination, papules on the dorsal surface of the proximal interphalangeal joints, base of the nose, and lateral surface \\nof the right hallux were observed. Hyperemia was also identified on the back, and edema of the proximal and distal interphalangeal joints, wrists \\nand knees. Laboratory tests revealed ESR and ANA within normal limits. Tests were negative for Anti-Sm, Anti-RO, rheumatoid factor, Anti-CCP, \\nHIV, and HCV. Chest CT-scan showed right axillary lymph node enlargement, measuring 2.7 cm, atheromatous aorta, and streaks with scattered \\natelectasis in the lower lobe, without other changes. On total abdominal ultrasound, prostate enlargement was detected. Immunohistochemistry \\nof skin biopsy identified atypical acinar proliferation. Histopathology skin biopsy stained with hematoxylin-eosin (January 2023) showed a \\ndense histiocytic infiltrate. Detail of the histiocytic component displayed “ground glass” eosinophilic cytoplasm. Immunohistochemistry was \\nnegative for S100 and CD34 and positive for CD68. Final diagnosis was MRH. The patient underwent treatment with drugs. In conclusion, clinical \\nmanifestations, including skin and joint changes, with signs and symptoms of rheumatic disorders (e.g. dermatomyositis and rheumatoid arthritis) \\nsimilar to those of MRH are expected in rheumatology practice. However, knowledge about disease progression in MRH is limited. Therefore, this \\ncase report is described due to its rarity \\nKeywords: Non-Langerhans-Cell Histiocytosis, Pathology, Muticentric Reticulohistiocytosis, Rheumatological Diseases,\",\"PeriodicalId\":332557,\"journal\":{\"name\":\"Selcuk Tip Dergisi\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2024-03-25\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Selcuk Tip Dergisi\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.30733/std.2023.01716\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Selcuk Tip Dergisi","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.30733/std.2023.01716","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
A Rare Case of Multicentric Reticulohistiocytosis in An Elderly Male Patient
Multicentric reticulohistiocytosis (MRH) is a rare systemic proliferative granulomatous disease of non-Langerhans cells. The aim of this study is
to report a case of multicentric reticulohistiocytosis that is easily misdiagnosed in clinical practice. A 74-year-old man sought the Rheumatology
office in December 2022 with a main complaint of arthralgia for six months, associated with morning stiffness, joint swelling, and upper and lower
limb weakness. On physical examination, papules on the dorsal surface of the proximal interphalangeal joints, base of the nose, and lateral surface
of the right hallux were observed. Hyperemia was also identified on the back, and edema of the proximal and distal interphalangeal joints, wrists
and knees. Laboratory tests revealed ESR and ANA within normal limits. Tests were negative for Anti-Sm, Anti-RO, rheumatoid factor, Anti-CCP,
HIV, and HCV. Chest CT-scan showed right axillary lymph node enlargement, measuring 2.7 cm, atheromatous aorta, and streaks with scattered
atelectasis in the lower lobe, without other changes. On total abdominal ultrasound, prostate enlargement was detected. Immunohistochemistry
of skin biopsy identified atypical acinar proliferation. Histopathology skin biopsy stained with hematoxylin-eosin (January 2023) showed a
dense histiocytic infiltrate. Detail of the histiocytic component displayed “ground glass” eosinophilic cytoplasm. Immunohistochemistry was
negative for S100 and CD34 and positive for CD68. Final diagnosis was MRH. The patient underwent treatment with drugs. In conclusion, clinical
manifestations, including skin and joint changes, with signs and symptoms of rheumatic disorders (e.g. dermatomyositis and rheumatoid arthritis)
similar to those of MRH are expected in rheumatology practice. However, knowledge about disease progression in MRH is limited. Therefore, this
case report is described due to its rarity
Keywords: Non-Langerhans-Cell Histiocytosis, Pathology, Muticentric Reticulohistiocytosis, Rheumatological Diseases,