Histiocytosis

Introduction:Langerhans cell histiocytosis (LCH) refers to a group of rare reticuloendothelial system disorders and it occurs most often in young adults and children. A 57-year-old edentulous female patient who complained of dull pain in the posterior region of the mandible referred to the dental office, with a complaint of dull pain in the posterior region of the mandible. The lesion was diagnosed as LCH . Oral manifestations could be the first signs of Langerhanschr('39') cell histiocytosis . Therefore, t he dentist must be aware of the oral symptoms so in order that the disease is not overlooked .

Erdheim-Chester disease (ECD) is a rare non-Langerhans histiocytosis characterized by systemic inflammation and granulomatous infiltration of multiple organs including the central nervous system (CNS), bones, and retroperitoneum. CNS infiltration occurs in one third of patients, but cognitive changes are common in patients without CNS disease. Here we investigate whether there is a neuroanatomic basis to observed cognitive deficits, even in absence of CNS disease. We present a volumetric analysis of eleven ECD patients without CNS tumors or prior neurotoxic treatments. Compared to age-matched controls, ECD patients have diffuse, bihemispheric reduction in cortical thickness and subcortical gray matter. These findings provide the first corroborating evidence for neurologic disease in ECD patients without direct CNS infiltration.

Langerhans cell histiocytosis is a benign histiocytic disorder touching both genders and can occur at any age. It is currently classified by the Histiocyte Society as an inflammatory myeloid neoplasm of mixed cellularity. Clinically, it is illustrated by single or multiple osteolytic bone lesions associated with ulceration of the skin and soft tissues. Disease outcome is highly variable, depending on the degree of involvement. Bone pathologies are observed in 60% of cases as uni-or multifocal lesions. Several treatment modalities have been proposed and include surgical excision, intralesion steroid injection, chemotherapy, and low-dose radiotherapy. In this paper, we report a case of a 42-year-old male suffering from gingival swelling in the left side of his mandible and the right side of the maxilla. Clinical, radiological, and histological examinations confirm the diagnosis of Langerhans cell histiocytosis. Hematological investigation, entire body CT scan, and bone scintigraphy confirmed the limitation of the lesions in the right maxilla and on the left mandible. The lesions were treated with fractionated stereotactic low-dose radiotherapy, 24 Gy in 16 fractions, by using a linear accelerator with a custom-made rigid mask for accurate immobilization of the head with confirmed precision, which allows noninvasive approaches. Complete remission was achieved clinically and radiologically after one year, and a panoramic X-ray after 5 years confirms the bone healing process. Fractionated stereotactic low-dose RT could be adopted as an effective treatment.

We present a 47-year-old man with a sudden eruption of more than 100 reddish-brown papules, which histologically exhibited a dense dermal proliferation of large mononuclear cells with vesicular nuclei and abundant pale cytoplasm. Electron microscopy and immunohistochemistry revealed findings consistent with indeterminate cell histiocytosis and the patient responded well to treatment with narrowband UVB therapy.

Lysosomal storage diseases (LSDs) are a group of heterogeneous disorders caused by defects in lysosomal enzymes or transporters, resulting in accumulation of undegraded macromolecules or metabolites. Macrophage numbers are expanded in several LSDs, leading to histiocytosis of unknown pathophysiology. Here, we found that mice lacking the equilibrative nucleoside transporter 3 (ENT3) developed a spontaneous and progressive macrophage-dominated histiocytosis. In the absence of ENT3, defective apoptotic cell clearance led to lysosomal nucleoside buildup, elevated intralysosomal pH, and altered macrophage function. The macrophage accumulation was partly due to increased macrophage colony-stimulating factor and receptor expression and signaling secondary to the lysosomal defects. These studies suggest a cellular and molecular basis for the development of histiocytosis in several human syndromes associated with ENT3 mutations and potentially other LSDs.