No abstract available.
Histiocytosis ; Histiocytosis, Non-Langerhans-Cell ; Stomach* ; Xanthomatosis*

No abstract available.
Histiocytosis, Non-Langerhans-Cell ; Xanthomatosis

Histiocytosis is an uncommon disease characterized by excessive accumulation of histiocytes. Here, we report a rare case of non-Langerhans-cell histiocytosis in a 51-year-old woman who presented with severe symptoms of pericardial effusion. Radiologic investigation also detected multiple bone (lower limbs, vertebrae, ribs, and ilium) lesions. Resected pericardium showed abundant mono- or multi-nucleated non-foamy histiocytes (CD68⁺/CD163⁺/S-100⁺/CD1α⁻/langerin⁻) in a fibroinflammatory background. The histiocytes demonstrated emperipolesis of lymphocytes, a hallmark feature of Rosai-Dorfman disease (RDD). However, molecular analysis revealed a BRAF V600E mutation of the proliferating histiocytes, highlighting the neoplastic features frequently observed in another non-Langerhans-cell histiocytosis known as Erdheim-Chester Disease (ECD). We consider this case to be a unique presentation of ECD harboring some RDD-like cells with emperipolesis, but not a case of RDD with a BRAF mutation concerning its clinical manifestation (involvement of the heart and bones) and neoplastic features.
Emperipolesis* ; Erdheim-Chester Disease* ; Extremities ; Female ; Heart* ; Histiocytes ; Histiocytosis ; Histiocytosis, Non-Langerhans-Cell ; Histiocytosis, Sinus ; Humans ; Lymphocytes ; Middle Aged ; Pericardial Effusion ; Pericardium ; Ribs ; Spine

Adult ; Histiocytosis, Non-Langerhans-Cell ; Humans ; Male

Juvenile xanthogranuloma (JXG) is a benign and self-limiting non-Langerhans-cell histiocytosis that generally occurs during infancy and childhood. It develops frequently in the head and neck but is very rare in the nasal cavity. To date, only five cases of JXG in the nasal cavity have been reported. Here, we report the second case of JXG in the nasal cavity in Korea. A 19-year-old male patient presented with a protruding 1.1 cm mass in the left nasal vestibule. Histologically, a dense dermal infiltrate of histiocytes with Touton giant cells was observed. Immunohistochemically, the histiocytes tested positive for CD68 and the S-100 protein but negative for CD1a. This shows that a S-100-positive histiocytic lesion dose not exclude a diagnosis of JXG.
Diagnosis ; Giant Cells ; Head ; Histiocytes ; Histiocytosis, Langerhans-Cell ; Histiocytosis, Non-Langerhans-Cell ; Humans ; Korea ; Male ; Nasal Cavity* ; Neck ; S100 Proteins ; Xanthogranuloma, Juvenile* ; Young Adult

Papular xanthoma is a newly classified type of normolipemic xanthoma that resembles juvenile xanthogranuloma and xanthoma disseminatum. Papular xanthoma is asymptomatic multiple yellowish papules with granulomatous lesion composed of foam cells and few giant cells, with scarce inflammatory cell infiltration. We report a case of papular xanthoma in a 3-year-old girl who showed typical clinical manifestation. She had multiple discrete, 0.2 to 0.5cm sized elevated dome-shaped yellowish papules on the trunk and face for 2years. Histopathologic findings showed granulomatous changes mainly composed of foam cells and few histiocytes, in the dermis.
Child, Preschool ; Dermis ; Female ; Foam Cells ; Giant Cells ; Histiocytes ; Histiocytosis, Non-Langerhans-Cell ; Humans ; Xanthogranuloma, Juvenile ; Xanthomatosis*

We made a clinical study on 10 cases of histiocytosis syndrome who had been admitted to the pediatric department of Soon Chun Hyang University Hospital from Jan. 1982 to Dec. 1991. The results were obtained as follows 1) The sex incidence revealed male predominance with the ratio 4:1. 2) Among 10 cases, 4 cases were classified as eosinophilic granuloma, 1 case as Letterer-Siwe disease, 1 case as linfection associated hemophagocytic syndrome and 4 cases as malignant histiocytosis. 3) The mean age of symptom onset was 4 and 7/12 years in all disease group. 4) The common clinical symptoms and signs at dignosis were dyspnea, mass, pain on lower extremities and fever. 5) The most common organ involved among 9 organ systems was liver-spleen and the number of organ systems involved were 1 in 3 2 ases(30%), 3 in 2 cases(20%), 8 in 2 cases (20%) and 6, 7 and 10 organs in each 1 case. 6) The abnormal hematologic findings (Hb; 10g/dl and/or WBC; 4,000/mm3 and/or PLT; 100,000/mm3) were found in 6 cases. 7) Low serum albumin was found in 2 cases. Results of blood culture were Pseudomonas aeruginosae and Salmonella typhi in each 1 case. 8) The common findings on tissue biopsy were histiocytic proliferation and infiltration. 9) 4 patients of MH who recieved chemotherapy, a combination of adriamycin, vincristine, cyclophosphamide and prednisone were given in a total of four courses every 2 weeks ad induction therapy. When complete response was attained, a combination of adriamycin, vincristine, prednisone (AOP) and cyclophosphamide, vincristine, prednisone(COP) was administered alternately every 4 weeks as maintenance therapy for 6-64 months. 10) Among 4 patiens of MH who recieved chemotherapy, 1 patient was lost during induction chemotherapy, for 1 day.2 patients expired during induction chemotherapy, for 1 month, 1 patient expired during maintenance chemotherapty, for 8months, Eosinophilic granuloma cases (3) were recieved currettage and no recurrence. IAHS case due to typhoid fever was improved spontaneously.
Biopsy ; Cyclophosphamide ; Doxorubicin ; Drug Therapy ; Dyspnea ; Eosinophilic Granuloma ; Fever ; Histiocytic Sarcoma ; Histiocytosis* ; Histiocytosis, Langerhans-Cell ; Humans ; Incidence ; Induction Chemotherapy ; Lower Extremity ; Lymphohistiocytosis, Hemophagocytic ; Male ; Prednisone ; Pseudomonas aeruginosa ; Recurrence ; Salmonella typhi ; Serum Albumin ; Typhoid Fever ; Vincristine

Reticulohistiocytosis represents a rare spectrum of non-Langerhans cell histiocytosis: the solitary cutaneous form (reticulohistiocytoma), the diffuse or multiple cutaneous form without systemic involvement, and multicentric reticulohistiocytosis with systemic involvement. To the best of our knowledge, there have been relatively few reports in the literature of solitary cutaneous reticulohistiocytoma found on the lip. We report here on a case of solitary cutaneous reticulohistiocytoma in a 58-year-old male, who presented with an asymptomatic dome-shaped nodule adjacent to the upper lip. The histopathologic examination revealed a dense dermal infiltrate, that was composed predominantly of large oncocytic histiocytes and multinucleated giant cells with abundant granular eosinophilic cytoplasms, which had a "ground-glass" appearance.
Cytoplasm ; Eosinophils ; Giant Cells ; Histiocytes ; Histiocytosis ; Histiocytosis, Non-Langerhans-Cell* ; Humans ; Lip* ; Male ; Middle Aged

Xanthoma disseminatum (XD) is one of cutaneous nonhistiocytosis X, and characterized by multiple, widely distributed red, brown, yellowish papules and nodules that tend to coalesce. We present a case of XD with characteristic clinical findings and treated with the combination therapy of COz laser vaporization and high dose steroid.
Histiocytosis, Non-Langerhans-Cell* ; Laser Therapy ; Lasers, Gas* ; Volatilization* ; Xanthomatosis*

Reticulohistiocytosis represents a rare spectrum of non-Langerhans cell histiocytosis: the solitary cutaneous form (reticulohistiocytoma), the diffuse cutaneous form without systemic involvement, and multicentric reticulohistiocytosis with systemic involvement. We report a case of solitary cutaneous reticulohistiocytosis in a 46-years-old male, who presented with an asymptomatic firm, dome-shaped nodule on the right vestibule of nose. The lesion was completely excised and no evidence of recurrence was observed.
Histiocytosis, Non-Langerhans-Cell ; Humans ; Male ; Nose ; Recurrence