1/19. Pagetoid self-healing Langerhans cell histiocytosis in an infant.We report Langerhans cell (LC) histiocytosis in a male infant who developed numerous papular lesions on the trunk and posterior scalp soon after birth and spontaneously recovered from the disease within 7 months. Histologically S-100-positive cells were detected in the epidermis and papillary dermis, in some lesions mostly in the epidermis. Tumor cells in the epidermis were either clustered, forming nests, or scattered singly in pagetoid fashion. Electron microscopy confirmed the presence of Birbeck granules in these cells. They exhibited many interesting features usually not found in normal LCs, including mitosis, frequent apoptosis, Birbeck granules invaginated in the nucleus, autophagocytosis of Birbeck granules, and active ingestion of extracellular material through Birbeck granules attached to cell membranes. It is suggested that either a strong epidermotropism of tumor cells or a proliferation of the resident LCs of the epidermis is responsible for this intraepidermal growth pattern. Cellular necrosis through very active apoptosis and the superficial nature of the growth might have contributed to the self-healing course in this patient.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/19. Mandibular histiocytosis X and acute lymphoblastic leukemia.The occurrence together of two major distinct diseases in the oral area is relatively rare. In this report, a case is presented in which histiocytosis X was found to develop in a child with acute lymphoblastic leukemia. The nature of the differentiated histiocytoses is explored, and the possibility of the relationship between a lymphoproliferative and a histiocytic disease entity is investigated.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/19. Sinus histiocytosis with massive lymphadenopathy and spinal epidural involvement: a case report and review of the literature.A case of "sinus histiocytosis with massive lymphoadenopathy" (SHML) in which spinal epidural involvement caused paraplegia is described. The literature pertaining to this newly recognized entity is reviewed; this review indicates that the process is not necessarily restricted to the lymph nodes and may be progressive in nature. The case we report is the first with histologically proved bone involvement.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/19. Langerhans cell histiocytosis in a child while in remission for acute lymphocytic leukemia.The occurrence of Langerhans cell histiocytosis (LCH) and malignancy in the same patient is rare. When LCH occurs concomitantly with acute leukemia, distinct temporal patterns often exist; acute myelogenous leukemia (AML) typically succeeds LCH, whereas acute lymphocytic leukemia (ALL) usually precedes it. We report a case of LCH developing in a child while in remission for ALL. Unique features of this case include the disseminated nature of the LCH and the death of the patient from LCH rather than ALL.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/19. Langerhans cell histiocytosis of the skull complicated with an epidural hematoma.Langerhans cell histiocytosis (LCH) is a rare disorder that affects the pediatric population. LCH complicated with a neurologic deficit due to the presence of epidural involvement is a rare condition. We describe the CT imaging features in a 2-year-old boy who presented with drowsy consciousness resulting from an epidural hematoma caused by spontaneous bleeding in an LCH of the skull. CT is an excellent means of depicting the full extent of bony destruction and the nature of the process.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/19. Multifocal eosinophilic granuloma ("hand-Schuller-Christian disease"). Report illustrating H-S-C chronicity and diagnostic challenge.We have described an unusual case of multifocal eosinophilic granuloma ("hand-Schueller-Christian disease") in a middle-aged woman. The case underscores the varied and subtle nature of the disease presentation and the extent to which many organ systems may become involved. Unusual features of her case include atypical bone roentgenograms, cutaneous anergy, panhypopituitarism and evidence of diffuse central nervous system dysfunction. Several features of multifocal eosinophilic granuloma present in the older age group are different from those presenting in children and young adults. Finally, multifocal eosinophilic granuloma may present all the clinical and laboratory features of a progressive, chronic disease.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
7/19. Generalized eruptive histiocytosis.The histiocytic disorders can be broadly categorized into histiocytosis X (Langerhans cell-derived) and non-X types. There are several variants of non-X histiocytoses that tend to occur in a generalized distribution on the body; these include xanthoma disseminatum, generalized eruptive histiocytosis (GEH), progressive nodular histiocytosis, and multicentric reticulohistiocytosis. Clinical and pathologic correlation are required for differentiating among these 4 disorders. We report a case of a middle-aged man in whom small, scattered, symmetrical lesions on the trunk and proximal extremities developed that, after correlating with biopsy specimen and laboratory results, were best classified as a non-X histiocytosis with features of GEH. GEH is a rare generalized non-X histiocytosis that occurs mainly in adults. It is characterized by multiple, scattered, symmetric lesions on the trunk and proximal extremities that are benign in nature and tend to resolve spontaneously. Recent literature has suggested that GEH may be a part of a continuous spectrum of non-X histiocytic disorders.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
8/19. Progressive form of pulmonary Langenharns' cell histiocytosis in a female adult non-smoker.Little is known about the cause, nature, treatment and prognosis of pulmonary Langerhans' cell histiocytosis (LCH) in adults. We report the case of a 44-year-old female non-smoker suffering from pulmonary histiocytosis who after a 7-year remission period relapsed with both lung and bone disease. Using a combination of corticosteroids, methotrexate and bone irradiation treatment, the patient achieved total disease remission. The patient was a non-smoking female who has had long-term and swift remission of the disease on two occasions.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
9/19. Difficulties in the diagnosis of rare immunological diseases manifesting with cystic lung disease and spontaneous pneumothorax: case reports.Pulmonary lymphangioleiomyomatosis (LAM) is a rare disease of an unknown cause affecting women of reproductive age and characterised by smooth muscle proliferation along lung lymphatic channels. pneumothorax develops in up to 80% of patients with LAM and may be the presenting manifestation of the disease. pneumothorax also precedes or complicates the clinical course of 25% of patients with Langerhans'-cell histiocytosis (LCH, histiocytosis X) pathologically characterised by involved tissue infiltration with large numbers of unusual Langerhans' cells, often organised as granulomas. A 41-year-old female patient was treated twice by simple tube drainage due to left pneumothorax in 1996. She was then diagnosed with chronic obstructive lung disease demonstrating with dyspnea, cough and wheeze. Abnormalities found in the high-resolution computed tomography (HRCT) scanning were characteristic of LAM with thin-walled parenchymal cysts distributed homogeneously in both lungs and with thickening of interlobular septa. A 38-year-old man was hospitalised due to chronic lung failure in the course of LCH characterised by small, poorly limited nodular lesions and thin-walled cysts revealed in HRCT scans. For two years of observation, he has suffered five episodes of right pneumothorax. When diagnosing pneumothorax in the middle-age patients, the possible cause of the disease is LAM or LCH. The use of HRCT scanning may enable good determination of the nature and distribution of parenchymal abnormalities found in the diseases.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
10/19. Langerhans cell histiocytosis involving epiphysis of a long bone.Eosinophilic granulomas of long tubular bones, a form of Langerhans cell histiocytosis, occurs in metaphyses and diaphyses with equal frequency. Epiphyseal location is unusual, with only 13 cases previously reported in the literature. The present case involves the epiphysis of the upper end of the left femur in a 12-year-old boy with pain and limp in the affected area. Radiographic findings were an oval, radiolucent rarefaction with nonsclerotic border, measuring approximately 3.5 x 3.0 cm. Microscopic examination identified aggregates of histiocytes, multinucleated giant cells, scattered eosinophils, and few plasma cells and lymphocytes. Ultrastructural studies demonstrated Birbeck granules within cytoplasm of histiocytes diagnostic of Langerhans cell histiocytosis. Whether Langerhans cell histiocytosis is a neoplastic disorder or a reactive process remains controversial. The true nature of the Langerhans cell, the proliferating cells and hallmark of this disease, is likewise under scrutiny.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
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