Erdheim-Chester Disease (ECD) is a rare condition and has various differential diagnoses with other forms of histiocytosis, classified as one of non-Langerhans histiocytosis. The diagnosis of this condition remains ch...Erdheim-Chester Disease (ECD) is a rare condition and has various differential diagnoses with other forms of histiocytosis, classified as one of non-Langerhans histiocytosis. The diagnosis of this condition remains challenging because its presentation includes non-specific systemic manifestations that can affect different organs caused by deposition of lipids and fibrosis. Most common include bone pain followed by progressive weakness and different lung manifestations. This case is about a rare presentation of ECD with Langerhans Histiocytosis as overlap syndrome, with findings of both diseases in a middle aged woman that presented dyspnea as the first symptom. The patient was treated initially as heart failure and remained without any improvement, being admitted to investigate. After a stricted follow-up, bone and lung involvement were noticed and a skin biopsy unveiled xanthomatized macrophages accompanied by Touton giant cells. This condition remains an important clinical entity and should provide new insights for clinicians dealing with respiratory diseases.展开更多
Erdheim-Chester disease(ECD) is a rare inflammatory syndrome in which systemic infiltration of non-Langerhans cell histiocytes occurs in different sites. Both the etiology and pathophysiology of ECD are unknown, but C...Erdheim-Chester disease(ECD) is a rare inflammatory syndrome in which systemic infiltration of non-Langerhans cell histiocytes occurs in different sites. Both the etiology and pathophysiology of ECD are unknown, but CD68 positive CD 1a/S100 negative cells are characteristic. The presentation of ECD differs according to the involved organs. This case report describes a patient with ECD and the gastrointestinal manifestations and unique endoscopic appearance as seen in gastroscopy and colonoscopy with histological proof of histiocyte infiltration of the lamina propria. The clinical and endoscopic findings of this unique case, to our knowledge, were never described before, so were the features of the gastrointestinal involvement in this disease.展开更多
Erdheim-Chester disease(ECD) is an uncommon, nonfamilial, non-Langerhans cell histiocytosis, which involves skeletal system and soft tissue usually in middle aged and elderly patients. The characteristic radiologic fe...Erdheim-Chester disease(ECD) is an uncommon, nonfamilial, non-Langerhans cell histiocytosis, which involves skeletal system and soft tissue usually in middle aged and elderly patients. The characteristic radiologic features include bilateral, symmetric cortical osteosclerosis of the diaphyseal and metaphyseal parts of the long bones, or bilateral symmetrically abnormal intense 99 mTechnetium labelling of the metaphyseal-diaphyseal region of the long bones, and computed tomography scan findings of "coated aorta" or "hairy kidneys". ECD in childhood with osteolytic lesion is extremely rare. We describe an unusual case with an expansile lytic bone lesion at presentation in a case of acute lymphoblastic leukemia.展开更多
Purpose: To describe a case of Erdheim-Chester disease with bilateral orbital involvement.Methods: A 43-year-old female with bilateral proptosis was presented. Its clinical features, image findings, pathological ch...Purpose: To describe a case of Erdheim-Chester disease with bilateral orbital involvement.Methods: A 43-year-old female with bilateral proptosis was presented. Its clinical features, image findings, pathological character and therapeutic effect were evaluated.Results: CT demonstrated bilateral, diffuse orbital mass. Histopathologic assessment revealed a diffuse xanthogranulomatous process with clusters of lipidladen histocytes. Numerous Touton giant cells were scattered throughout the lesion. Renal and heart failure happened during a 6-year follow-up period. Long bones roentgenogram demonstrated diffuse symmetrical sclerosis with extensive, lytic lesions. Systemicadministration of corticosteroids, chemotherapy, immunoglobulin and traditional Chinese medicine showed good therapeutic result. Conclusions: An administration of systemic corticosteroids, chemotherapy, immuno- globin and traditional Chinese medicine can control Erdheim-Chester disease. Further exploration of its pathogenesis and collection of useful clinical data are required.展开更多
文摘Erdheim-Chester Disease (ECD) is a rare condition and has various differential diagnoses with other forms of histiocytosis, classified as one of non-Langerhans histiocytosis. The diagnosis of this condition remains challenging because its presentation includes non-specific systemic manifestations that can affect different organs caused by deposition of lipids and fibrosis. Most common include bone pain followed by progressive weakness and different lung manifestations. This case is about a rare presentation of ECD with Langerhans Histiocytosis as overlap syndrome, with findings of both diseases in a middle aged woman that presented dyspnea as the first symptom. The patient was treated initially as heart failure and remained without any improvement, being admitted to investigate. After a stricted follow-up, bone and lung involvement were noticed and a skin biopsy unveiled xanthomatized macrophages accompanied by Touton giant cells. This condition remains an important clinical entity and should provide new insights for clinicians dealing with respiratory diseases.
文摘Erdheim-Chester disease(ECD) is a rare inflammatory syndrome in which systemic infiltration of non-Langerhans cell histiocytes occurs in different sites. Both the etiology and pathophysiology of ECD are unknown, but CD68 positive CD 1a/S100 negative cells are characteristic. The presentation of ECD differs according to the involved organs. This case report describes a patient with ECD and the gastrointestinal manifestations and unique endoscopic appearance as seen in gastroscopy and colonoscopy with histological proof of histiocyte infiltration of the lamina propria. The clinical and endoscopic findings of this unique case, to our knowledge, were never described before, so were the features of the gastrointestinal involvement in this disease.
文摘Erdheim-Chester disease(ECD) is an uncommon, nonfamilial, non-Langerhans cell histiocytosis, which involves skeletal system and soft tissue usually in middle aged and elderly patients. The characteristic radiologic features include bilateral, symmetric cortical osteosclerosis of the diaphyseal and metaphyseal parts of the long bones, or bilateral symmetrically abnormal intense 99 mTechnetium labelling of the metaphyseal-diaphyseal region of the long bones, and computed tomography scan findings of "coated aorta" or "hairy kidneys". ECD in childhood with osteolytic lesion is extremely rare. We describe an unusual case with an expansile lytic bone lesion at presentation in a case of acute lymphoblastic leukemia.
文摘Purpose: To describe a case of Erdheim-Chester disease with bilateral orbital involvement.Methods: A 43-year-old female with bilateral proptosis was presented. Its clinical features, image findings, pathological character and therapeutic effect were evaluated.Results: CT demonstrated bilateral, diffuse orbital mass. Histopathologic assessment revealed a diffuse xanthogranulomatous process with clusters of lipidladen histocytes. Numerous Touton giant cells were scattered throughout the lesion. Renal and heart failure happened during a 6-year follow-up period. Long bones roentgenogram demonstrated diffuse symmetrical sclerosis with extensive, lytic lesions. Systemicadministration of corticosteroids, chemotherapy, immunoglobulin and traditional Chinese medicine showed good therapeutic result. Conclusions: An administration of systemic corticosteroids, chemotherapy, immuno- globin and traditional Chinese medicine can control Erdheim-Chester disease. Further exploration of its pathogenesis and collection of useful clinical data are required.
