No abstract available.
Bone Marrow* ; Dermatoglyphics* ; Histiocytes*

Langerhans cell histiocytosis (LCH) encompasses a wide range of clinical presentation and its clinical course varies widely from spontaneous regression to severe disseminated disease with the risk of permanent consequences. Although pathogenesis of LCH has been a conundrum, recent advances have led to a better understanding of the molecular pathogenesis of the disease. Especially, advanced genomic analyses have suggested that LCH is a disorder of MAPK pathway mutations. Optimal treatment for LCH has not been established yet. Histiocyte Society has launched a new international multi-center clinical trial, LCH-IV to solve remaining major issues in the treatment of LCH. The aim of this review is to provide an overview of recent understandings on the pathogenesis and treatment of LCH.
Histiocytes ; Histiocytosis, Langerhans-Cell

Malignant fibrous histiocytoma is uncommon pleomorphic tumor of the soft tissues possibly arising from the histiocyte. A case of malignant fibrous histiocytoma of the retroperitoneum stimulated our interest in this uncommon and pathologically intriguing lesion. So we report a case of malignant fibrous histiocytoma in the retroperitoneal space.
Histiocytes ; Histiocytoma ; Histiocytoma, Malignant Fibrous* ; Retroperitoneal Space

We report a case of multiple xanthogranuloma occurring ori the scalp in a 33 years old wornan. Multiple yellowish papules were developed on her scalp 3 months ago. Histopathologic findings showed massive granulomatous infiltrations of foamy histiocytes Touton giant cells in the dermis, and these cells were positive for lipid stain. This case was considered to be multiple xanthogranuloma in an adult, and treated by sur- gical excision.
Adult* ; Dermis ; Giant Cells ; Histiocytes ; Humans ; Scalp

Adult xanthogranuloma is nearly indistinguishable from juvenile xanthogranuloma characterized by yellow-red nodules on the skin, normal lipid findings and the proliferation of xanthomatizied histiocytes with foamy Touton cells, histologically. But unlike juvenile xanthogranuloma, there is no visceral involvement, spontaneous regression and more solitary lesions. Multiple adult xanthogranulomas are rare entities that present more spontaneous involution. Here we report a case of a 39-year-old patient with multiple xanthogranulomas which developed on the face, trunk and extremities. Diagnostic workup revealed normal lipid profile and no extracutaneous manifestations.
Adult* ; Extremities ; Histiocytes ; Humans ; Skin ; Xanthogranuloma, Juvenile

A xanthogranuloma is a self-limiting, benign disorder that is caused by the proliferation of histiocytes. It usually develops on the face, scalp, and upper extremities, but rarely develops in the ear. Only two cases of xanthogranuloma of the ear have been reported in the literature. We present a xanthogranuloma on the concha of the Lt. ear in a 14-year-old boy.
Adolescent ; Ear ; Histiocytes ; Humans ; Scalp ; Upper Extremity

Malignant fibrous histiocytoma is an uncommon pleomorphic tumor of the soft tissues possibly arising from the histiocyte. A case of huge malignant fibrous histiocytoma weighing 10.2kg in the retroperitoneal space is reported with brief review of literatures.
Histiocytes ; Histiocytoma ; Histiocytoma, Malignant Fibrous* ; Retroperitoneal Space

No abstract available.
Bone Marrow* ; Histiocytes* ; Niemann-Pick Diseases*

Rosai-Dorfman disease (RDD) is a rare benign proliferative disorder of histiocytes in the lymph nodes with or without extranodal involvement. RDD limited to the skin without nodal involvement, known as cutaneous Rosai-Dorfman disease, is very rare. We describe a 34-year-old female with RDD of the skin over the chest. A large nodule with satellite lesions was excised for histopathological examination. Microscopically, there were many large histiocytes (Rosai-Dorfman cells) exhibiting emperipolesis, among many plasma cells, lymphocytes and neutrophils throughout the dermis and the subcutaneous tissue. The histiocytes were immunohistochemically positive for S-100 protein but negative for CD 1a. Physical examination showed no lymphadenopathy or any extra-cutaneous lesions. Serological tests indicated a past infection with Epstein-Barr virus and cytomegalovirus. The diagnosis of cutaneous RDD may be difficult in the absence of associated lymphadenopathy or any specific features of the skin lesion. Hence, not only is histopathological examination required for definitive diagnosis but a high index of suspicion by the clinicians and pathologists is essential to help diagnose this very rare disease.
seconds ; Cutaneous ; Histiocytes ; Enlargement of lymph nodes ; Diagnostic

A 33-year-old male had a 22-year history of generalized xanthogranulomas but had a normal lipid profile. He also developed ulcerating plaques on his arm and back that were consistent with cutaneous tuberculosis on histopathologic and polymerase chain reaction PCR studies. In a normolipemic patient with generalized xanthogranulomas, a reactive granulomatous response of histiocytes to infection is explored since these share a common CD 14+ precursor with the macrophages that are vital in tuberculosis.