Cases reported "Xanthomatosis"

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1/32. poems syndrome with xanthomatous cells. Polyneuropathy Organomegaly Endocrinopathy M-protein skin changes.

    A diffuse xanthomatous infiltration was detected on a biopsy of the hyperpigmented patches in a 40-year-old man with POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, skin changes) syndrome. Multiple biopsies of the hyperpigmented patches showed diffuse or perivascular foamy histiocytes in the upper and mid dermis, a sparse infiltrate of lymphocytes, fibrosis, hyperpigmentation of the basal layer, and capillary proliferation. The foamy cells were positive for periodic acid-Schiff (PAS) with diastase, oil red-O, and CD68. To our knowledge, this is the first case with foamy histiocytes without xanthomatous-appearing skin lesions in poems syndrome. It would be worth searching for xanthomatous histiocytes in the hyperpigmented skin of patients with poems syndrome.
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2/32. Guess what! SALT - related B-cell lymphoma presenting as a xanthomatous infiltration of the neck.

    A 37-year-old man without previous medical history working as a lock keeper was seen in our unit for a progressive painless subcutaneous flesh coloured infiltration of the lower anterior area of the neck growing slowly over 5 months. Clinical cutaneous findings showed a non pruriginous yellowish papulonodular eruption mimicking xanthomas (Figs. 1 and 2). No other clinical abnormalities were found. A cutaneous biopsy specimen was performed. Histopathological examination revealed, under a normal epidermis, a dense lymphoplasmocytoid infiltrate involving the dermis with periadnexal and perivascular reinforcement (Figs. 3 and 4).
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3/32. necrobiotic xanthogranuloma presenting as a solitary tumor.

    We report on a 60-year-old overweight white woman who presented with an asymptomatic flat, hard, yellow-brown subcutaneous plaque on her right hip. A total excision was performed. Histopathologic examination showed all the major features of a necrobiotic xanthogranuloma (NX) involving the entire dermis and subcutis, including areas of necrobiosis with cholesterol clefts, granulomatous infiltrate with some bizarre giant cells, numerous Touton cells, foamy cells, lymphoid follicles with germinal centers, foci of plasma cells, and "Touton cell panniculitis." A laboratory investigation revealed only slightly increased titers of cholesterol, strong positivity of anti-borrelia antibodies, and diffuse skeletal osteoporosis with fractures of seven vertebrae. After 4 years of close follow-up, the osteoporosis was improved, and there were no signs of paraproteinemia, malignancy, or new skin lesions. The authors suggest that this case could represent a solitary clinical variant of NX without paraproteinemia.
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4/32. CD30 (Ki-1)-positive large-cell cutaneous T-cell lymphoma with secondary xanthomatous changes after radiation therapy.

    A 72-year-old woman with a large mass on her forehead was found to have primary cutaneous CD30-positive large T-cell lymphoma and was treated with radiation therapy. Five weeks after the completion of radiation therapy, a large, yellow plaque developed on the forehead. Histopathologic examination showed numerous xanthoma-like cells with clear and foamy cytoplasm that infiltrated the upper and mid dermis with no residual lymphoma. To our knowledge, this is the first report of xanthomatous change after radiation therapy for primary cutaneous CD30-positive large cell lymphoma. We believe that the xanthomatous change after radiation therapy probably was caused by lysis of radiosensitive lymphoma cells and histiocytic scavenging of lipid-membrane debris.
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5/32. Normolipemic plane xanthoma associated with adenocarcinoma and severe itch.

    Normolipemic plane xanthomas are yellow-red-colored flat patches or plaques with barely palpable borders, under normolipemic conditions usually involving the eyelids, the lateral sides of the neck, the upper aspect of the trunk, or the flexural folds. Histologically the lesions are characterized by an infiltrate consisting of foamy macrophages in the papillary and middermis with a distinct perivascular localization. Plane xanthoma has been associated with monoclonal gammopathy, cryoglobulinemia, and myeloproliferative disorders. We present a patient in whom plane xanthoma developed on the upper aspect of the back, which was accompanied by severe itch in the affected area. These symptoms started 1 month after resection of an adenocarcinoma of the rectum that was complicated by recurrent abdominal abscesses and, currently, by ongoing inflammatory bowel disease. A hypothetic pathophysiologic scheme of events leading to xanthoma formation in this patient is presented.
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6/32. Immunohistochemical study of human xanthoma.

    Specimens of human tuberous xanthomas obtained from two hyper-beta-lipoproteinemic patients were prepared with HE and sudan III stain and by direct immunofluorescence with fluorescein isothiocyanate labeled antihuman beta-lipoprotein rabbit serum. In some histiocytes and foam cells in early stages, lipids were stained in granular patterns and specific fluorescence was observed. In some foam cell nests, specific fluorescence was weak or absent, and orange-colored lipid autofluorescence was present. The foam cells in such nests were probably late foam cells and corresponded to the large cytoplasmic foam cells stained densely and amorphously for lipids. It was concluded that at early stages of xanthoma formation, serum lipoproteins were incorporated into dermal histiocytes and early foam cells. The apoproteins of the incorporated lipoproteins were probably degraded and lipid residues accumulated in the cells. Specimens of tuberous xanthoma covered by plane xanthoma showed lipids and bright specific fluorescence at the basement membrane zone. A faint fluorescence was observed in the upper dermis and even in parts of the epidermal intercellular spaces.
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7/32. The Koebner phenomenon in eruptive xanthoma.

    A 49-year-old woman with Type V hyperlipidemia had yellow papules smaller than 3 mm in diameter with red haloes on her elbows, shoulder, buttocks, and hands. Two linear lesions composed of papules arising at the sites of skin previously injured by a cat scratch were observed. Histopathological examination of these papules revealed the presence of many foam cells in the dermis which stained positively with oil red O stain. A survey of the literature found only 5 cases of the Koebner phenomenon in xanthoma.
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8/32. Verruciform xanthoma in association with discoid lupus erythematosus.

    Verruciform xanthoma (VX) is an uncommon lesion occurring primarily in the oral cavity. Cutaneous lesions are much less common and they preferentially arise on anogenital skin. They are not necessarily associated with a pre-existing inflammatory process. We report a VX in association with a long-standing lesion of discoid lupus erythematosus (DLE) on the scalp of a 34-year-old black woman. This association, which to our knowledge has not been previously reported, is consistent with the proposed pathogenetic mechanism of entrapment and subsequent degeneration of epithelial cells in the papillary dermis of VX. Histological distinction of VX from squamous cell carcinoma, with which this lesion may be clinically confused, is straightforward.
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9/32. Verruciform xanthoma in close association with isolated epidermolytic acanthoma: a case report and review of the Japanese dermatological literature.

    A 52-year-old man presented to our department with a scrotal skin nodule, first noted as a papule two to three years previously. The nodule was red and pedunculated with a granular surface and a diameter of 10 mm. Three red papules were scattered around the nodule. Histopathologic examination of the nodule showed epidermal papillary hyperplasia, collections of foam cells in the papillary dermis, and a dense infiltration of inflammatory cells into all dermal layers. In addition, granular degeneration was seen in the pedunculated lesion of the nodule free from the foam cells. Microscopic examination of the red papules also showed granular degeneration. The patient was diagnosed with verruciform xanthoma associated with isolated epidermolytic acanthoma. This is the first report of these two lesions occurring at the same site on the scrotum.
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10/32. A case of diffuse plane normolipemic xanthomatosis associated with pancytopenia and monoclonal gammopathy.

    We report a case of diffuse plane normolipemic xanthomatosis (DPNX) which showed poorly demarcated, uncommon, yellow macules symmetrically distributed on the nape, axillae and inguinal folds accompanied by severe, persistent itching. Histopathological and ultrastructural studies of skin biopsy specimens revealed the existence of some foamy cells and the deposition of neutral fat in the upper papillary dermis. Laboratory investigations and bone marrow aspirate smears showed that our patient had myelodysplastic syndrome (MDS) associated with pancytopenia and monoclonal gammopathy of undetermined significance. Because our patient had neither a malignant hematological disorder nor a severe systemic disease, monoclonal gammopathy might explain the pathogenesis of DPNX in the present case.
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