1/64. Verruciform xanthoma associated with squamous cell carcinoma.Verruciform xanthoma (VX) is a rare lesion of unknown etiology that is typically solitary and predominantly located within the oral cavity. Less commonly, they arise on the skin, with the majority of cases occurring in anogenital sites. They can be confused clinically with verruca vulgaris, condyloma, leukoplakia, verrucous carcinoma, and squamous cell carcinoma. Histologic features include acanthosis with uniform elongation of the rete ridges and xanthomatous cells that lie in and are typically confined to the papillary dermis. Although epidermal atypia is not a characteristic finding, we describe an unusual case of VX that has features of both VX and squamous cell carcinoma. In addition, there was a VX with typical histologic characteristics located at a separate site in the same patient. This case is also the first to our knowledge to be reported on the neck and axilla and is the third case associated with cutaneous graft versus host disease secondary to bone marrow transplant for acute lymphoblastic leukemia.- - - - - - - - - - ranking = 1keywords = xanthoma (Clic here for more details about this article) |
2/64. Benign cephalic histiocytosis progressing into juvenile xanthogranuloma: a non-Langerhans cell histiocytosis transforming under the influence of a virus?Benign cephalic histiocytosis (BCH) is best understood as a form of non-Langerhans cell histiocytosis, specifically as an early mononuclear variant of juvenile xanthogranuloma (JXG). However, the progression of BCH into JXG in the same patient has only been reported once before. We describe the case of a 2-year-old girl with asymptomatic, large, ill-defined infiltrated flat plaques over both cheeks, in addition to isolated papules. A punch biopsy of a plaque revealed dermal infiltration by vacuolated and scalloped histiocytes positive for CD68 KP-1, and that lacked expression of CD1a and S-100 protein, favoring macrophages over langerhans cells. Electron microscopy study showed comma-shaped intracytoplasmic bodies in the histiocytic cells leading to the diagnosis of BCH. One year later, after an episode of varicella-zoster infection, the flat plaques over the cheeks became large reddish-yellow nodules, and in a second biopsy appeared to progress to JXG. Virus-related mechanisms of progression are discussed.- - - - - - - - - - ranking = 1.6490342899361keywords = histiocytosis (Clic here for more details about this article) |
3/64. Juvenile xanthogranuloma of the nasal cavity.Juvenile xanthogranuloma is a benign, normolipaemic, self-healing condition and a type of histiocytosis that occurs most frequently in infants and children. This condition usually presents with solitary or multiple cutaneous lesions and occasionally with visceral lesions. We report a case of juvenile xanthogranuloma occurring in the nasal cavity. We believe this is the first report, in the English literature, of juvenile xanthogranuloma occurring in this site.- - - - - - - - - - ranking = 0.16490342899361keywords = histiocytosis (Clic here for more details about this article) |
4/64. September 2000: 15 year old girl with intracranial lesion.The September COM: We reported the case of a 15 year old white girl with solitary brain cortical lesion clinically mimicking meningioma. Microscopic examination revealed histiocytic lesion with foamy and giant cells predominately of Touton type. Subsequent clinical examination did not reveal any similar lesion in the skin or in other location. Expression of S-100 protein in histiocytes is unusual finding, but has been reported recently in juvenile xanthogranulomas (JXG). The only specific ultra-structural finding differing JXG from histiocytic neoplasm of Langerhans' cells histiocytosis type seems to be Birbeck granules.- - - - - - - - - - ranking = 0.16490342899361keywords = histiocytosis (Clic here for more details about this article) |
5/64. Intracardiac juvenile xanthogranuloma in a newborn.Juvenile xanthogranuloma (JXG) presents a normolipemic non-langerhans cells histiocytosis. JXG usually presents with cutaneous lesions. Visceral involvement is rare but may affect various organs. Deep JXG differs histologically from the cutaneous form by its tendency to consist solely of homogeneous proliferation of histiocytes without any xanthomatous or Touton giant cells. awareness of the possibility of this atypical presentation of JXG helps in making the correct histologic diagnosis, which is supported by proving adequate immunomarkers on histiocytes (mainly PG-M1, an antibody against the CD68 antigen). JXG may present with intramuscular lesions only; however, rarely JXG has been reported to affect the heart but not without the typical cutaneous manifestations. We present an unusual case of deep JXG without systemic disease or metabolic abnormalities. To our knowledge, this is a first reported case of intracavitar JXG without skin lesions.- - - - - - - - - - ranking = 0.33157009566028keywords = xanthoma, histiocytosis (Clic here for more details about this article) |
6/64. Histopathologic maturation of juvenile xanthogranuloma in a short period.We present a case of solitary juvenile xanthogranuloma (JXG) on the scalp of an 8-month-old girl. The initial biopsy specimen showed a dense collection of small histiocytes as evidenced by CD68 staining without either lipidization or giant cell formation, admixed with a small number of lymphocytes. On the other hand, sections from the excised specimen obtained 2 weeks after the initial biopsy from the same site showed a mixed proliferation of abundant foam cells together with Touton giant cells, some small histiocytes, and small numbers of lymphocytes and eosinophils. Mitotic figures were fewer in the excised nodule than in the initial biopsy specimen. Fascicles of spindle-shaped cells arranged in a vague storiform pattern were additionally found in the deep portion of the nodule. Our case findings suggest that xanthomatization of the JXG could have been accelerated by the inflammation associated with the biopsy, based on the histopathologic fact that the change from an early phase to a mature form occurred within the very short period of 2 weeks.- - - - - - - - - - ranking = 0.16666666666667keywords = xanthoma (Clic here for more details about this article) |
7/64. Juvenile xanthogranuloma as a sequel to Langerhans cell histiocytosis: a report of three cases.We report three children who had multisystem Langerhans cell histiocytosis (LCH) with cutaneous involvement and subsequently developed juvenile xanthogranuloma (JXG). JXG appeared 3--6 years after the initial manifestation of LCH. JXG lesions, which presented as yellowish papules, revealed typical Touton giant cells and were factor xiiia positive but S100 and CD1a negative. Non-LCH histiocyte disorders, such as JXG, are known to occur as a reaction to a variety of external stimuli such as infection and trauma. It is therefore conceivable that the inflammatory reaction associated with LCH may have precipitated the development of JXG in our patients. Alternatively, one could speculate that this association might be due to a common histogenetic precursor of the cell types involved.- - - - - - - - - - ranking = 0.82451714496805keywords = histiocytosis (Clic here for more details about this article) |
8/64. Cellular pathology of homozygous familial hypercholesterolemia.tissues were studied from four subjects with homozygous familial hypercholesterolemia (FH). The specimens consisted of tissues obtained from a 20-week-old fetus at autopsy, samples from a 9-year-old girl during open-heart surgery, and biopsies of cutaneous xanthomas from a 13-year-old girl and a 21-year-old man. The FH fetus, but not the 3 control fetuses, exhibited multifocal lipid deposition particularly involving the stromal cells of the thymus, spleen, and skin and both the stromal and parenchymal cells of the kidney. Only one minute focus of intimal lipid accumulation was found in the aorta and coronary arteries of the FH fetus. A segment of the ascending aorta from the 9-year-old girl showed: 1) foam-cell transformation of many medial smooth-muscle cells, 2) abnormal vascularization of the inner media and intima, and 3) intimal involvement by a typical artherosclerotic plaque with lipid deposits in thin, elongated cells that showed some myocytic features and in foam cells that lacked such features. The mitral and aortic valves of this patient also contained numerous foam cells and showed mild to moderate fibrous thickening. A segment of the saphenous vein, however, contained no lipid deposits. The three xanthomas from two FH homozygotes exhibited marked lipid accumulation in histiocytic foam cells but no lipid deposits in the endothelium of blood vessels in the lesions. The findings in this study, in conjunction with those reported in studies of other FH homozygotes, indicate that homozygous FH is characterized by accelerated atherosclerosis and prominent lipid accumulation in macrophages and other stromal cells of the aortic and mitral valves, skin, tendon, and, varibly, in other extravascular sites. Since most of the intracellular lipid was in the form of non-membrane-bound neutral lipid droplets, it appears that the cytoplasm is the major site of lipid storage in this disease.- - - - - - - - - - ranking = 0.33333333333333keywords = xanthoma (Clic here for more details about this article) |
9/64. Bilateral multifocal uveal juvenile xanthogranuloma in a young boy with systemic disease.BACKGROUND: Juvenile xanthogranuloma is a rare non-Langerhans cell histiocytosis that could occasionally produce diffuse systemic disease in young children, but associated posterior uveal lesions are very uncommon. methods: Case report of an 8-month-old boy with macrocephaly who presented with chronic subdural haematoma and intracranial hypertension. A combination of nodular skin lesions and bilateral yellowish choroidal infiltration with limited retinal involvement suggested juvenile xanthogranuloma. RESULTS: The diagnosis was confirmed by a skin biopsy, and oral corticosteroid therapy was introduced. Progression of the disease to involve an anterior uveitis with hypopyon and numerous other systemic lesions, including the central nervous system, lung, liver, spleen, kidney and testis, was also suggestive of Letterer-Siwe disease. histiocytes were negative for Langerhans cell markers (S-100 and CD1a) and positive for macrophage marker (CD68). Electron microscopy failed to show Birbeck granules. Ocular lesions regressed under prolonged corticosteroid treatment, but resurgence of the other lesions required chemotherapy with vinblastine. CONCLUSION: In this atypical systemic variant of juvenile xanthogranuloma with bilateral uveal involvement, the immunohistochemical and ultrastructural findings were crucial in distinguishing juvenile xanthogranuloma from Langerhans cell histiocytosis.- - - - - - - - - - ranking = 0.32980685798722keywords = histiocytosis (Clic here for more details about this article) |
10/64. Primary cerebral non-Langerhans cell histiocytosis: MRI and differential diagnosis.We report a young woman with primary cerebral non-Langerhans cell histiocytosis of the juvenile xanthogranuloma family. The clinical course was complicated by extensive infiltration of cranial nerves and meninges and epi- and intramedullary spinal dissemination. Whereas the cutaneous form of juvenile xanthogranuloma is usually benign and self-limited, central nervous system involvement is associated with high morbidity and mortality and might therefore be considered a separate clinical entity.- - - - - - - - - - ranking = 0.82451714496805keywords = histiocytosis (Clic here for more details about this article) |
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