A case study of sinus histiocytosis of Rosai-Dorfman (SH) clinically limited to the skin is presented with immunohistochemical study of the infiltrate, in both paraffin and cryostat sections. Factor XIIIa, a dendrocyte marker, was demonstrated in the cytoplasm of histiocytes. This feature had not been previously reported in this disease. In addition, the cells expressed S100 protein, CD4, CD1a,...

Langerhans cell histiocytosis is a disease which results from accumulation or proliferation of a clonal population of cells with the phenotype of Langerhans cells arrested at an early stage of activation that are functionally defi cient. The etiology and pathogenesis of the disorder are still unknown. There are ongoing investigations to determine whether it is a reactive or a neoplastic disease...

Accepted 29 March 1996 In 1953, Lichtenstein introduced the unifying term 'histiocytosis X' to embrace several previous eponyms, including eosinophilic granuloma, Letterer-Siwe disease and Hand-Schuller-Christian disease.' In 1987, a more prominent designation, Langerhans cell histiocytosis (LCH), was recommended to replace histiocytosis X.' The term is more appropriate as it reflects the fact ...

BACKGROUND Crystal storing histiocytosis (CSH) is a rare disorder characterized by accumulation of histiocytes containing crystalline material inclusions. This entity can be misdiagnosed as infection or tumor. We present a case of idiopathic CSH mimicking metastatic squamous cell carcinoma in a cervical lymph node and review of literature regarding this rare entity. METHODS AND RESULTS The pa...

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-...

Congenital self-healing reticulohistiocytosis, also known as Hashimoto-Pritzker disease, is a single system Langerhans cell histiocytosis that typically presents in healthy newborns and spontaneously regresses. In the present report, we described a 2-month-old Thai female newborn with multiple hypopigmented flat-topped papules without any internal organ involvement including normal blood cell c...

Histiocytic syndromes in children are divided into 3 classes: (i) Langerhans-cell histiocytosis (LCH) or Histiocytosis-X (HX), (ii) Hemophagocytic syndromes—Familial erythrophagocytic lymphohistiocytosis (FEL) and infection-associated hemophagocytic syndrome (IAHS) due to viruses (VAHS), and occasionally bacterial, fungal or parasitic, and (iii) Malignant histiocytic disorders(l). The lesional ...

Langerhans cell histiocytosis (LCH) is an infrequent disorder complex. It is a disease of myeloid dendritic cells, lymphocytes, and macrophages mixed with eosinophils and neutrophils [1]. The accumulation of these cells causes the classic lytic bone lesions, skin rashes, lymphadenopathy, and organ dysfunction. Langerhaus cell histocytosis occurs at all ages from infancy up to senility with the ...

A 22-year-old man was referred to our institution due to lower back pain and was diagnosed with Langerhans cell histiocytosis of the thoracic and lumbar spine. The patient achieved complete remission with radiotherapy and chemotherapy. One year later, right cervical lymphadenopathy was observed and Hodgkin's lymphoma was confirmed on biopsy. The patient was treated with chemotherapy and autolog...

Division and proliferation of dendritic cells (DCs) have been proposed to contribute to homeostasis and to prolonged antigen presentation. Whether abnormal proliferation of dendritic cells causes Langerhans cell histiocytosis (LCH) is a highly debated topic. Transgenic expression of simian virus 40 (SV40) T antigens in mature DCs allowed their transformation in vivo while maintaining their phen...