网状组织细胞增生症组织病理及免疫组化研究

观察１例多中心网状组织细胞增生症和５例网状组织细胞肉芽肿（ＲＨ）病理表现，两病均由甲状腺嗜酸细胞样单核组织细胞和多核组织细胞组成，在ＲＨ还可见空泡状、棘状和黄瘤样单核组织细胞。免疫组化示：ＫＰ１（ＣＤ６８）、溶菌酶、ａ１－抗胰蛋白酶、花生凝集素和Ｖｉｍｅｎｔｉｎ阳性，Ｍａｃ（３８７），Ｓ－１００，ＨＬＡ－ＤＲ、Ｄｅｓｍｉｎ、Ａｃｔｉｎ阴性，讨论两病的组织病理、免疫组化鉴别及ＲＨ与成人黄色肉芽肿关系。     

Multicentric reticulohistiocytosis with extra-mammillary Paget’s disease: a case report

Multicentric reticulohistiocytosis (MRH) is a very rare systemic disease with variable phenotypic presentation and a high rate of misdiagnosis. Here we describe a patient with MRH and extra-mammillary Paget’s disease (EMPD), a diagnosis that has not previously been described in the literature.     

伴广泛系统受累的多中心网状组织细胞增生症

报告1例伴广泛系统受累的多中心网状组织细胞增生症。患者女,33岁。关节畸形2年,四肢、耳、口腔黏膜结节,臀部红色斑块1年,呼吸困难6个月,声音嘶哑3个月。耳和左前臂的结节活检,组织病理学检查显示,真皮内大量组织细胞和多核巨细胞,胞质嗜酸性,呈“毛玻璃”样。支气管镜检查示支气管内多发结节。纤维喉镜检查示左劈裂处1.5cm×2.0cm大赘生物。胸部磁共振成像(MRI)示大量胸腔积液和心包积液。腹部超声检查示脾大和腹水。经泼尼松、环磷酰胺和甲氨蝶呤联合治疗后皮肤和关节症状显著改善。     

Multicentric reticulohistiocytosis in a patient with severe preeclampsia

A multigravida patient with polyarthralgia and eruptions on the head and fingers was seen at 6 weeks’ gestation. No histological examination was performed before the current pregnancy. She developed severe early onset preeclampsia associated with swelling of the knees and increased cutaneous nodules, biopsies of which revealed multicentric reticulohistiocytosis. At 28 weeks’ gestation an elective cesarean section was performed and a 580-g male infant was delivered. Received: June 1999 / Accepted: 23 September 1999     

Multicentric reticulohistiocytosis associated with ovarian cancer

Multicentric reticulohistiocytosis (MR) is an uncommon disease characterized by joint and cutaneous manifestations. The diagnosis must be confirmed by histological evidence of typical histiocytes and multinucleated giant cells. Many conditions, including malignancy, have been described in association with MR. We herein report a female case of MR in whom partial improvement was obtained by steroid and low-dose methotrexate treatments. However, ovarian cancer was found and therefore a surgical resection and chemotherapy were performed. These treatments resulted in the complete resolution of the skin and joint symptoms. These findings support the close linkage between MR and malignancy and the efficacy of cytotoxic drugs for the treatment of MR.     

多中心网状组织细胞增生症1例

报告1例多中心网状组织细胞增生症。患者女,52岁。关节疼痛1年,头面、耳、手背、口腔黏膜出现黄红色丘疹结节伴发热半年。手背结节活检,组织病理学检查显示,真皮内大量组织细胞和多核巨细胞,胞质嗜酸性,呈“毛玻璃”样。免疫组化染色CD68(+)。诊断为多中心网状组织细胞增生症,经泼尼松、羟基氯喹和阿司匹林联合治疗后皮肤和关节症状显著改善,正在随访中。     

Lipoiddermatoarthritis (multizentrische Reticulohistiocytose)

Zusammenfassung An Hand einer eigenen Beobachtung, einem 10jährigen Mädchen, wird die Lipoiddermatoarthritis (multizentrische Reticulohistiocytose) beschrieben. Diese stellt ein eigenständiges, seltenes Krankheitsbild dar, das mit Knötcheneruptionen an der Haut und Schleimhaut und einer chronischen Polyarthritis einhergeht. Biochemisch lassen sich keine kennzeichnenden Befunde erheben. Histologisch bestehen die intracutan gelegenen Knötchen aus einem dichten Infiltrat aus histiocytären Elementen und mehrkernigen Riesenzellen, in deren Cytoplasma Neutralfett, Phospholipide und Glykoproteine gespeichert sind. In der Synovia der betroffenen Gelenke finden sich gleichartige Knötchen. Die therapeutisch bislang noch unbeeinflußbare Krankheit führt zu schweren Gelenksdestruktionen. Pathogenetisch wird eine intracelluläre Stoffwechselstörung des histiocytären Mesenchyms angenommen, deren Ätiologie noch unbekannt ist.

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Lipoid dermatoarthritis (multicentric reticulohistiocytosis)

The case of a 10-year-old girl with an infrequently observed lipoid dermatoarthritis is reported. This is a systemic disease of unknown etiology characterized by a nodular eruption of the skin, mucosa and synovia, resulting in destructive arthritis and disfigurement of the facies. Biopsy of a nodule, whether from skin or synovia, discloses a cellular infiltrate consisting of histiocytes and multinucleated giant cells with lipid inclusions. No effective treatment is known.

     

Cutaneous histiocytoses in children

Cutaneous histiocytoses constitute a heterogeneous group of diseases characterised by the cutaneous accumulation of cells with the cytological and phenotypic features of macrophages or dendritic cells. The clinical spectrum ranges from self-resolving, skin-limited conditions to severe, multiorgan disease with a high morbidity rate. Until recently, cutaneous histiocytoses were classified according to the immunophenotype of the pathological cells, with differentiation between Langerhans cell histiocytosis (LCH) [CD1a+, CD207 (langerin)+] and non-Langerhans cell histiocytosis (CD68+, CD163+, CD1a?, CD207?). Over the last 12 years, a number of new pathophysiological findings (in particular, molecular pathology results) regarding histiocytoses have contributed to a new classification based on molecular alterations, as well as on clinical and imaging characteristics and the phenotype. The most frequent entities in children are juvenile xanthogranuloma and LCH.     

Extracellular signal‐regulated kinase activation of self‐healing Langerhans cell histiocytosis: A case report

A 3‐month‐old boy developed small papules on his trunk. After the papules increased in number, the patient was diagnosed with Langerhans cell histiocytosis based on the pathological findings. He was referred to our department for further examination. Upon initial examination, the papules and nodules were scattered on his back, abdomen and lumbar region. Because he did not present with any organ involvement except the skin, he was diagnosed with single‐system and skin‐limited Langerhans cell histiocytosis. Skin rashes were treated with a topical steroid and started regressing 3 months after onset. All papules disappeared 6 months after onset. In this boy, the Langerhans cell histiocytosis tumor cells expressed phosphorylated extracellular signal‐regulated kinases. In Langerhans cell histiocytosis, BRAF V600E and other genes are known to mutate to act as driver mutations in stem cells of the myeloid dendritic cell lineage. Consequently, extracellular signal‐regulated kinases are continuously activated, which contributes to Langerhans cell histiocytosis carcinogenesis.