Humans ; Histiocytosis, Sinus/diagnosis* ; Diagnosis, Differential ; Lung

Humans ; Histiocytosis, Sinus/diagnosis* ; Fractures, Compression/complications* ; Diagnosis, Differential

Diagnosis, Differential ; Histiocytosis, Sinus/diagnostic imaging* ; Humans ; Skin Diseases/diagnostic imaging*

Rosai-Dorfman disease is a rare histiocytic disorder, clinically characterized by massive, bilateral painless cervical lymphadenopathy with potential for extranodal manifestations. We report a 45-year-old male patient who presented with a slowly growing erythematous nodule of the left chin. The mass appeared non-vascular on computed tomography study, but ultrasonogram was suggestive of a vascular lesion. The lesion was excised with presumptive diagnosis of a hemangioma. However, histopathologic examination of the surgical biopsy revealed histiocytic infiltration with emperipolesis, which was pathognomic for Rosai-Dorfman disease. Additional imaging studies did not reveal lymph node enlargement or other extranodal manifestation. The patient was diagnosed with cutaneous form of the Rosai-Dorfman disease and was discharged home. He remains free of local recurrence at 8 months.
Biopsy ; Chin ; Diagnosis ; Emperipolesis ; Hemangioma ; Histiocytosis, Sinus* ; Humans ; Lymph Nodes ; Lymphatic Diseases ; Male ; Middle Aged ; Recurrence ; Ultrasonography

Rosai-Dorfman disease is a rare, idiopathic, benign, and self-limiting histiocytic proliferative disorder. A 26-year-old man presented with a single massive cutaneous nodule (reaching 30 cm in diameter) on the left shoulder and back for 15 months. The routine hematological and biochemical tests were normal. Magnetic resonance scanning showed the lesion involved the skin, subcutaneous tissue, and subjacent muscle group, accompanied by obvious lymph node enlargement in the left part of the neck, supraclavicular fossa, and axillary fossa. The histopathology of the left cervical lymph node revealed diffuse effacement of the normal nodal architecture, with patchy chronic inflammatory cell infiltrates comprising lymphocytes and sheets of histiocytes. Some histiocytes contained lymphocytes within their pale cytoplasm. Many multinucleated giant cells were found; however, caseating granulomas were not seen. The skin and muscle biopsy specimen obtained from the back revealed infiltrating lymphocytes and histiocytes diffusely distributed in the dermis, subcutaneous tissue, and crevices of the muscle fibers. The phenomenon of emperipolesis and the presence of multinucleated giant cells were also seen. Immunohistochemical staining revealed that the histiocytes were positive for S-100 protein and CD68 but negative for CD1a. Immunophenotyping of the infiltrating lymphocytes indicated positive reactions to CD3, CD45RO, CD5, CD7, CD4, CD8 (partly), CD79a, CD20 (partly), and Ki-67 (<1%). The final diagnosis was Rosai-Dorfman disease. Owing to the extensive and deep involvement of the subcutaneous tissue and muscles, the patient did not undergo surgery to excise the massive skin nodule. The lesion showed no obvious change at the 12-month follow-up.
Adult ; Biopsy ; Cytoplasm ; Dermis ; Diagnosis ; Emperipolesis ; Follow-Up Studies ; Giant Cells ; Granuloma ; Histiocytes ; Histiocytosis, Sinus* ; Humans ; Immunophenotyping ; Lymph Nodes ; Lymphocytes ; Muscles ; Neck ; S100 Proteins ; Shoulder* ; Skin ; Subcutaneous Tissue

Rosai-Dorfman disease (RDD) is a benign proliferative histiocytic disorder of unknown etiology, which typically manifests as lymphadenopathy and systemic symptoms. Lymph node involvement is typical, but soft tissue RDD without nodal or systemic involvement is extremely rare. We report on a case of soft tissue RDD in a 16-year-old girl with a palpable mass on her buttock. It was firm, mobile, and discrete without tenderness. Excisional biopsy was performed, then RDD was confirmed histologically. RDD is a non-neoplastic disease that should be considered in the differential diagnosis of other soft tissue tumors. While the optimal treatment for extranodal RDD remains controversial, surgical excision is typically curative.
Adolescent ; Biopsy ; Buttocks ; Diagnosis, Differential ; Emperipolesis ; Female ; Histiocytosis, Sinus* ; Humans ; Lymph Nodes ; Lymphatic Diseases

Histiocytosis, Sinus ; diagnosis ; Humans

OBJECTIVETo study the prevalence of IgG4-positive plasma cells in Rosai-Dorfman disease and to assess the association between Rosai-Dorfman disease and IgG4-related sclerosing disease (IgG4-SD).

METHODSThe clinicopathologic features of 12 tissue samples of Rosai-Dorfman disease (11 extranodal and one nodal) from nine patients were reviewed. The degree of fibrosis and occlusive phlebitis was studied by HE staining. The expression of IgG4 and IgG in plasma cells were studied by immunohistochemistry (EnVision) and quantitatively analyzed by medical image analysis system.

RESULTSNine tissue samples showed different degree of fibrosis (four tissue samples were mild, one tissue sample was moderate and four tissue samples were severe) and two tissue samples showed occlusive phlebitis in the lesional tissue. Immunohistochemical study showed marked infiltration by IgG4-positive plasma cells (> 50 per high-power field) in four tissue samples, moderate infiltration (30 to 50 per high-power field) in two tissue samples, mild (10 to 29 per high-power field) in three cases and negative infiltration (< 10 per high-power field) in three tissue samples (P < 0.01). Three tissue samples fulfilled the diagnostic criteria of IgG4-SD (> 50 IgG4-positive plasma cells per high-power field and IgG4-to-IgG ratio > 40%), including one tissue sample each of Rosai-Dorfman disease in the left facial skin, above the left eye socket, and in the right parotid.

CONCLUSIONSSome cases of Rosai-Dorfman disease fulfill the diagnostic criteria and show the histologic features of IgG4-SD. They may represent members of the IgG4-SD spectrum. The detection of IgG4-positive plasma cells in the lesional tissues of Rosai-Dorfman disease may have clinical pathological significance.

Fibrosis ; Histiocytosis, Sinus ; diagnosis ; immunology ; Humans ; Immunoglobulin G ; chemistry ; Immunohistochemistry ; Phlebitis ; pathology ; Plasma Cells ; chemistry

Rosai-Dorfman disease (RDD) is an idiopathic histioproliferative disorder of lymph node and extranodal site. Bone involvement is very rare. We report a case of extranodal RDD of the tibia in 32-year old male. The patient presented with pain with no evidence of lymphadenopathy. Clinico-radiologic diagnosis was metastatic carcinoma or Langerhans cell histiocytosis, but, histopathologic examination confirmed the diagnosis with RDD. We performed curettage on the osteolytic lesion of tibia. In South Korea, there was no report about RDD of the extremity and we want to report this case with review of the literature.
Curettage ; Diagnosis ; Extremities ; Histiocytosis, Langerhans-Cell ; Histiocytosis, Sinus* ; Humans ; Korea ; Lymph Nodes ; Lymphatic Diseases ; Male ; Tibia*

Adrenal Cortex Hormones ; therapeutic use ; Adult ; Antitubercular Agents ; therapeutic use ; Female ; Histamine Antagonists ; therapeutic use ; Histiocytosis, Sinus ; diagnosis ; drug therapy ; Humans ; Hyperplasia ; diagnosis ; drug therapy ; Langerhans Cells ; drug effects ; pathology ; Prednisone ; therapeutic use ; Young Adult