Brain Diseases ; pathology ; Histiocytosis, Sinus ; pathology ; Humans ; Skull ; pathology

Histiocytosis, Langerhans-Cell ; pathology ; Humans ; Intestinal Diseases ; pathology

Adult ; Female ; Histiocytosis, Sinus ; pathology ; surgery ; Humans ; Skull ; pathology

Histiocytosis, Langerhans-Cell ; Humans ; Infant ; Male ; Temporal Bone ; pathology

Female ; Histiocytosis, Langerhans-Cell ; pathology ; Humans ; Infant, Newborn

Although Rosai-Dorfman disease is a rare disorder characterized by proliferation of distinctive histiocytes within lymph node sinuses and lymphatics, sometimes involving extranodal sites, the nasal cavity and pharyngeal cavity are rarely involved additional. In this report, a 22-year old man with multiple subcutaneous lumps and submucosal lumps in bilateral nasal cavity and pharyngeal cavity is presented, which was diagnosed by nasopharyngeal endoscope,computed tomography scan and biopsy. The subcutaneous lumps were ablated and prednisone was accepted for three months as preventive therapy.
Histiocytosis, Sinus ; pathology ; Humans ; Male ; Nasal Cavity ; pathology ; Pharynx ; pathology ; Subcutaneous Tissue ; pathology ; Young Adult

Encephalocele ; pathology ; Granulomatosis with Polyangiitis ; pathology ; Histiocytosis, Sinus ; pathology ; Humans ; Otorhinolaryngologic Diseases ; pathology ; Pathology ; trends ; Precancerous Conditions ; pathology ; Tuberculosis, Laryngeal ; pathology

By using the method of clonal analysis the evidence to prove that Hemophagocytic syndrome (HPS) is reactive or malignant was investigated to probe into the pathogenesis of HPS and its relations with clinical prognosis. The macrophages abnormally proliferated in bone marrow were isolated. Electrophoresis analysis was made after DNA extraction, enzyme restriction of human ardrogen receptor (HUMARA) genetic locus, and PCR amplification. In the 9 specimens, clonal proliferation was found in 2 cases and nonclonal proliferation in 7. Among the 7 cases of nonclonal proliferation, 3 were voluntarily discharged without clinical outcome, 2 cases fully recovered after 2-3 week treatment of large dose gamma globulin intravenous drip and hormone therapy, 1 case died at the 43th day after the hormone and anti-infection therapy, and one case was found to have granular leukoblast in peripheral blood after 3 weeks and diagnosed as having M2a after bone puncture. For the two patients with clonal proliferation, one obtained remission after chemotherapy and the other was died after 32 days without chemotherapy. It was concluded that there do exist clonal or malignant proliferation in HPS, so not every case is reactive.
Clone Cells ; Histiocytosis, Non-Langerhans-Cell/*blood ; Histiocytosis, Non-Langerhans-Cell/therapy ; Macrophages/*pathology ; gamma-Globulins/therapeutic use

Diagnosis, Differential ; Histiocytoma, Benign Fibrous ; metabolism ; pathology ; Histiocytosis, Langerhans-Cell ; metabolism ; pathology ; Histiocytosis, Sinus ; metabolism ; pathology ; surgery ; Humans ; Infant ; Male ; S100 Proteins ; metabolism ; Xanthogranuloma, Juvenile ; metabolism ; pathology

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 CD1a. 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.
Exanthema ; diagnosis ; pathology ; Female ; Histiocytosis, Sinus ; diagnosis ; pathology ; Humans ; Middle Aged ; Rosacea ; pathology