窦组织细胞增生伴巨大淋巴结病（sinus histiocytosis with massive lymphadenopathy,SHML）又称为Rosai—Dorfman病，是一种病因不明，罕见的良性淋巴组织增生性疾病，好发于儿童及青少年。因其累及部位不同而临床表现多样，无明确实...窦组织细胞增生伴巨大淋巴结病（sinus histiocytosis with massive lymphadenopathy,SHML）又称为Rosai—Dorfman病，是一种病因不明，罕见的良性淋巴组织增生性疾病，好发于儿童及青少年。因其累及部位不同而临床表现多样，无明确实验室指标支持该病，且属于儿科少见病，在儿科临床极易误诊和漏诊，本文对其诊断及治疗方法等进行归纳总结，以提高对该疾病的认识。展开更多
A case of cutaneous Rosai-Dorfman disease （CRDD） presenting as a granulomatous rosacea-like rashs was reported. A 45-year-old Chinese woman presented with a 1-month history of a widespread nonpruiginous papulonodula...A case of cutaneous Rosai-Dorfman disease （CRDD） presenting as a granulomatous rosacea-like rashs was reported. A 45-year-old Chinese woman presented with a 1-month history of a widespread nonpruiginous papulonodular eruption. The rash had begun on her face and rapidly progressed to involve the neck and extremities. She was otherwise healthy, with no history of fever, malaise, or weight loss. Physical examination revealed multiple symmetrically distributed discrete and coalescing red plaques, papules and nodules scattered over the face, neck and extremities. No appreciable lymphadenopathy or hepatosplenomegaly was noted. There was no mucosal involvement. The biopsy specimen obtained from the face demonstrated the epidermis was normal, while the superficial dermis contained sheets of histiocytes with abundant, focally foamy cytoplasm. The histiocytes were surrounded by a patchy lymphocytic and plasma cell infiltrate. There was no significant histiocytic atypia. Some of these histiocytes engulfed, without destroying, lymphocytes and neutrophils （emperipolesis）. Immunohistochemical staining revealed that the histiocytes were strongly positive for S100 protein, weakly positive for CD68, and negative for CDla. A diagnosis of CRDD was made. Oral prednisone therapy was initiated at a dosage of 30 mg/d for 3 weeks and then tapered over the ensuing 2 weeks. After 5 weeks of treatment, the lesions had markedly improved.展开更多
Rosai Dorfman disease, also known as histiocytosis with massive lymphadenopathy, is a very rare idiopathic disease. It is characterized by over production and accumulation of non Langerhans sinus histiocyte most often...Rosai Dorfman disease, also known as histiocytosis with massive lymphadenopathy, is a very rare idiopathic disease. It is characterized by over production and accumulation of non Langerhans sinus histiocyte most often in lymph nodes, but may occur in other areas leading to organ damage. Based on the research, it can be caused by an infectious agent, immunodeficiency or autoimmunity and genetic causes. Extranodal manifestation is uncommon, however extranodal sites include liver, kidney, respiratory organs, orbit and eyeball. We present a case report of a 44-year-old female with recurrent bilateral orbital infiltration as first location of Rosai Dorfman disease.展开更多
文摘窦组织细胞增生伴巨大淋巴结病（sinus histiocytosis with massive lymphadenopathy,SHML）又称为Rosai—Dorfman病，是一种病因不明，罕见的良性淋巴组织增生性疾病，好发于儿童及青少年。因其累及部位不同而临床表现多样，无明确实验室指标支持该病，且属于儿科少见病，在儿科临床极易误诊和漏诊，本文对其诊断及治疗方法等进行归纳总结，以提高对该疾病的认识。
文摘A case of cutaneous Rosai-Dorfman disease （CRDD） presenting as a granulomatous rosacea-like rashs was reported. A 45-year-old Chinese woman presented with a 1-month history of a widespread nonpruiginous papulonodular eruption. The rash had begun on her face and rapidly progressed to involve the neck and extremities. She was otherwise healthy, with no history of fever, malaise, or weight loss. Physical examination revealed multiple symmetrically distributed discrete and coalescing red plaques, papules and nodules scattered over the face, neck and extremities. No appreciable lymphadenopathy or hepatosplenomegaly was noted. There was no mucosal involvement. The biopsy specimen obtained from the face demonstrated the epidermis was normal, while the superficial dermis contained sheets of histiocytes with abundant, focally foamy cytoplasm. The histiocytes were surrounded by a patchy lymphocytic and plasma cell infiltrate. There was no significant histiocytic atypia. Some of these histiocytes engulfed, without destroying, lymphocytes and neutrophils （emperipolesis）. Immunohistochemical staining revealed that the histiocytes were strongly positive for S100 protein, weakly positive for CD68, and negative for CDla. A diagnosis of CRDD was made. Oral prednisone therapy was initiated at a dosage of 30 mg/d for 3 weeks and then tapered over the ensuing 2 weeks. After 5 weeks of treatment, the lesions had markedly improved.
文摘Rosai Dorfman disease, also known as histiocytosis with massive lymphadenopathy, is a very rare idiopathic disease. It is characterized by over production and accumulation of non Langerhans sinus histiocyte most often in lymph nodes, but may occur in other areas leading to organ damage. Based on the research, it can be caused by an infectious agent, immunodeficiency or autoimmunity and genetic causes. Extranodal manifestation is uncommon, however extranodal sites include liver, kidney, respiratory organs, orbit and eyeball. We present a case report of a 44-year-old female with recurrent bilateral orbital infiltration as first location of Rosai Dorfman disease.
