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 with generalized systemic involvement

We describe a 33-year-old woman with a 2-year history of rheumatoid arthritis-like joint changes and a 1-year history of papulonodules on the fingers, ears, oral mucosa, forearm, elbows and thighs, and erythematous plaques on the buttocks. Six months after the onset of the cutaneous lesions she had dyspnoea, 3 months later she developed a husky sound. Biopsies from the nodules on the ear and left forearm revealed multinucleated giant cells with eosinophilic 'ground-glass' cytoplasm. Bronchoscopy revealed that there were nodules in the bronchus. A biopsy taken from a nodule from the bronchus was consistent with multicentric reticulohistiocytosis. Fibrostic laryngoscopy showed a mass 1.5 x 2.0 cm(2) in size on the left aryepiglottic fold and posterior commissure. Magnetic resonance imaging revealed a large pleural effusion and pericardiac effusion. Ultrasound revealed splenomegaly and peritoneal fluid. Combination therapy with prednisone, cyclophosphamide and methotrexate significantly improved cutaneous and joint symptoms. The huge cutaneous erythematous plaques and the generalized systemic involvement make this case interesting.     

Multicentric reticulohistiocytosis

BACKGROUND: Multicentric reticulohistiocytosis (MR) is a rare histiocytic systemic disease mainly affecting the skin and synovia. OBJECTIVE: To present the main and newest aspects of MR. METHOD: A review of the current medical literature, composing a retrospective study of 96 case reports published world-wide since 1977. RESULTS: Facial and hand skin nodules with symmetric arthritis were the most frequent clinical presentation. The pathognomonic sign of'coral beads' and vermicular erythematous lesions bordering nostrils, seems to be very characteristic of MR. There is a significant association with cancer. Histiocytic cells of MR are best characterized on immunohistochemistry by its immunoreactivity for vimentin, CD68 and CD45 and non-reactivity for S-100 protein, CD34 and factor XIIIa. Although the outcome of the disease is usually very limiting for the patients, treatment with immunosuppressive drugs may be useful. CONCLUSION: MR is a unique histiocytic proliferative disease with a peculiar immunophenotypic pattern and distinctive clinical and histopathological aspects.     

Multicentric reticulohistiocytosis

A 41 -year-old man presented with multiple, painful and tender nodules on the palms, soles, scalp; and on the limbs predominantly around the joints with associated arthropathies. Smaller nodules were seen on the ear helices. There was no other clinically evident or investigative abnormality. Histopathological study confirmed a diagnosis of multicentric reticulo histiocytosis.     

Multicentric reticulohistiocytosis

A 54-year old male presented with multiple, itchy, reddish-brown coloured papules on the face, arms and trunk of 8 months duration. Histopathological study of the skin biopsy specimen revealed features typical of multicentric reticulohistiocytosis.     

Multicentric reticulohistiocytosis

A case of multicentric reticulohistiocytosis in a 49-year-old man associated with polymyositis is presented. The clinical features along with the light and electron microscopic studies are commented. Partial improvement of the skin lesions was achieved after treatment with azathioprine but the patient's general condition remained unchanged.     

Multicentric reticulohistiocytosis

Multicentric reticulohistiocytosis is a rare granulomatous disease of unknown etiology, characterized by cutaneous nodules and destructive arthritis. Skin lesions can cause significant deformity, and approximately half of affected patients develop a severe disabling arthritis. The disease is often associated with malignancy; however, the paraneoplastic nature of multicentric reticulohistiocytosis is not established. The diagnosis is confirmed by the presence of oncocytic (“ground-glass”) histiocytes and multinucleated giant cells on histopathology of the cutaneous nodules and the synovial membrane.     

Multicentric reticulohistiocytosis

A case of multicentric reticulohistiocytosis associated with a pleural mesothelioma is reported. The cutaneous and joint manifestations resolved after treatment with cyclophosphamide, and the remission persisted until the patient's death 3 years later. As corticosteroids have such an unpredictable effect on this condition, cyclophosphamide may be regarded as the treatment of choice until more cases can be assessed.     

Multicentric reticulohistiocytosis.

Multicentric reticulohistiocytosis is a rare disorder of the skin and joints that possesses distinctive histologic findings. A case is reported from shortly after its onset to a stage of resolution 4 1/2 years later.     

Multicentric reticulohistiocytosis with elevated cytokine serum levels

Multicentric reticulohistiocytosis (MRH) is an uncommon non-Langerhans cell histiocytosis of unknown etiology. It is a multisystem disorder characterised by a papulonodular skin eruption, mainly in the extensor surfaces, and destructive polyarthritis. Histologically, either cutaneous lesions or the synovium show a dense dermal infiltrate of histiocytes and multinucleated giant cells with an eosinophilic granular material in the cytoplasm. In the immunohistochemical analysis these cells stain positively with monocyte/macrophage markers (CD68 and CD45), as well as with certain cytokines (tumor necrosis factor-α, interleukin 1β and interleukin 6). Moreover, recent reports suggest an osteoclastic nature of the infiltrating cells, as they stain strongly with osteoclast tissue lytic markers including tartrate-resistant acid phosphatase and cathepsin K. We report a case of MRH presenting with clinical features of dermatomyositis. Furthermore, the patient showed elevated cytokine serum levels that lowered after therapy.     

Multicentric reticulohistiocytosis in a child

A case of multicentric reticulohistiocytosis in an 8-year-old girl, which is a diagnosis rarely seen in children, is presented. Multicentric reticulohistiocytosis is a disorder of unknown aetiology, predominantly affecting the joints, skin and mucosa. Joint symptoms, but not cutaneous lesions, have improved with treatment with methotrexate.     

Multicentric reticulohistiocytosis and malignant disease

Multicentric reticulohistiocytosis developed in a 45-year-old woman, who had a 3 year history of untreated breast carcinoma. A review of the literature revealed that malignant disease has been associated with 27% of reported cases. It is considered that, like adult dermatomyositis, the onset of multicentric reticulohistiocytosis should stimulate detailed investigation for an underlying neoplasm.     

Multicentric reticulohistiocytosis with lungs and liver involved

We describe a 56-year-old man with a 2-year history of papulonodules, pruritic and painful on palpation, on the head, trunk, limbs, buttocks and scrotum and a 1.5-year history of rheumatoid arthritis-like joint changes. Biopsies from the nodules on the head and left elbow revealed multinucleated giant cells with eosinophilic 'ground-glass' cytoplasm. Computed tomography revealed that there were scattered nodules in the liver and both lungs. Biopsies taken from nodules in the right lung and liver were consistent with multicentric reticulohistiocytosis. The widely scattered cutaneous papulonodules and the generalized systemic involvement make this patient interesting, and the condition should be differentiated from other diseases in clinicopathological practice.