BACKGROUND Rosai–Dorfman disease(RDD)is a rare histiocytic proliferation of unknown etiology commonly found in children and adolescents.The common manifestation of RDD is massive and painless bilateral cervical lymph...BACKGROUND Rosai–Dorfman disease(RDD)is a rare histiocytic proliferation of unknown etiology commonly found in children and adolescents.The common manifestation of RDD is massive and painless bilateral cervical lymphadenopathy with extranodal disease.While extranodal involvement in RDD is common,the spleen is an infrequent site of disease.CASE SUMMARY We report a 10-mo-old female infant with RDD presenting multiple splenic masses without cervical lymphadenopathy.She had fever,and blood tests showed leukocytosis,anemia,and elevated erythrocyte sedimentation rate and Creactive protein.Ultrasound,computed tomography,and magnetic resonance images demonstrated multiple splenic masses.Despite antibiotic therapy,her symptoms were not relived.She underwent diagnostic splenectomy and was discharged with recovery.CONCLUSION In pediatric patients with refractory infectious symptoms or hematological abnormalities,clinicians should suspect RDD,even in patients without significant lymphadenopathy.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
Background:Rosai-Dorfman disease (RDD) is an idiopathic histiocytic proliferative disorder characterized by sinus histiocytosis and massive lymphadenopathy. Extranodal and central nervous system (CNS) involvement is u...Background:Rosai-Dorfman disease (RDD) is an idiopathic histiocytic proliferative disorder characterized by sinus histiocytosis and massive lymphadenopathy. Extranodal and central nervous system (CNS) involvement is unusual. We describe two distinctly rare cases of isolated intradural RDD of the spine without cervical lymphadenopathy. Case presentation:We report our experience in the management of two patients with RDD. The first patient had two ventral intradural lesions, radiologically in favour of multiple spinal meningiomas, and the second patient presented an intradural extramedually lesion, extending into the right foramina, suggestive of a sacral perineural cyst. Treatments consisted of total excision and subtotal excision without steroids and radiotherapy, respectively. The definitive diagnosis was confirmed by histopathology and immunochemistry. Conclusions:RDD should be considered in the rare differential diagnosis of a spinal disease. Resection of the lesions is an effective treatment choice to relieve spinal compression symptoms. The efficacy of the adjuvant therapy to control the disease is unknown.展开更多
文摘BACKGROUND Rosai–Dorfman disease(RDD)is a rare histiocytic proliferation of unknown etiology commonly found in children and adolescents.The common manifestation of RDD is massive and painless bilateral cervical lymphadenopathy with extranodal disease.While extranodal involvement in RDD is common,the spleen is an infrequent site of disease.CASE SUMMARY We report a 10-mo-old female infant with RDD presenting multiple splenic masses without cervical lymphadenopathy.She had fever,and blood tests showed leukocytosis,anemia,and elevated erythrocyte sedimentation rate and Creactive protein.Ultrasound,computed tomography,and magnetic resonance images demonstrated multiple splenic masses.Despite antibiotic therapy,her symptoms were not relived.She underwent diagnostic splenectomy and was discharged with recovery.CONCLUSION In pediatric patients with refractory infectious symptoms or hematological abnormalities,clinicians should suspect RDD,even in patients without significant lymphadenopathy.
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Background:Rosai-Dorfman disease (RDD) is an idiopathic histiocytic proliferative disorder characterized by sinus histiocytosis and massive lymphadenopathy. Extranodal and central nervous system (CNS) involvement is unusual. We describe two distinctly rare cases of isolated intradural RDD of the spine without cervical lymphadenopathy. Case presentation:We report our experience in the management of two patients with RDD. The first patient had two ventral intradural lesions, radiologically in favour of multiple spinal meningiomas, and the second patient presented an intradural extramedually lesion, extending into the right foramina, suggestive of a sacral perineural cyst. Treatments consisted of total excision and subtotal excision without steroids and radiotherapy, respectively. The definitive diagnosis was confirmed by histopathology and immunochemistry. Conclusions:RDD should be considered in the rare differential diagnosis of a spinal disease. Resection of the lesions is an effective treatment choice to relieve spinal compression symptoms. The efficacy of the adjuvant therapy to control the disease is unknown.
