Presentation
The gross and microscopic pathology are presented with immunohistochemical, ultrastructural and biochemical details. [ncbi.nlm.nih.gov]
To the best of our knowledge, the present report is the only one that characterizes XD on a molecular level. [medicaljournals.se]
Hematological
- Hemophilia A
[…] sickness 【血栓症】*thrombosis 【血栓性静脈炎】*thrombophlebitis 【血栓性動脈内膜炎】*thromboendarteritis 【血栓塞栓症】*thromboembolism 【血痰】*bloody sputum *hemosputum *sputum cruentum[L] 【血鉄症】*hemosiderosis 【血糖症】*glycemia 【血尿】*hematuria *erythruria [赤血球尿症] *urina cruenta[L] 【血友病】*hemophilia [medo.jp]
Entire Body System
- Gaucher Disease
甲状腺機能亢進症 hyperthyroidism 甲状腺機能低下症 hypothyroidism 甲状腺中毒症 thyrotoxicosis 後天性免疫不全症候群 acquired immune deficiency syndrome 高コレステロール血症 hypercholesterolemia 高トリグリセリド血症 hypertriglyceridemia 高リポ蛋白血症 hyperlipoproteinemia ゴーシェ病 Gaucher disease 骨粗鬆症 osteoporosis [jsge.or.jp]
Jaw & Teeth
- Xerostomia
[…] subdural hemorrhage 【硬膜下膿瘍】*subdural abscess 【硬膜外血腫】*epidural hematoma 【硬膜外膿瘍】*epidural abscess (口) 【口蓋炎】*palatitis 【口蓋垂炎】*uvulitis *staphylitis 【口蓋垂裂】*cleft uvula *uvula bifida[L] 【口蓋乳頭嚢胞】*cyst of papilla palatina 【口蓋裂】*cleft palate *palatoschisis 【口腔乾燥症】*xerostomia [medo.jp]
Eyes
- Visual Impairment
Within 6 months increasing visual impairment of the right eye developed; a computed tomographic scan revealed multiple cerebral and cerebellar lesions. [ncbi.nlm.nih.gov]
- Visual Impairment
Within 6 months increasing visual impairment of the right eye developed; a computed tomographic scan revealed multiple cerebral and cerebellar lesions. [ncbi.nlm.nih.gov]
Skin
- Papule
Wikipedia: Xanthoma disseminatum a rare cutaneous condition that preferentially affects males in childhood, characterized by the insidious onset of small, yellow-red to brown papules and nodules that are discrete and disseminated. :717 It's a histiocytosis [memidex.com]
The clinical features subsequently changed to a generalized eruption of confluent, yellowish papules, and diabetes insipidus developed. [ncbi.nlm.nih.gov]
The patient was an 8‐year‐old boy who was admitted to our hospital because of red‐brown papules which developed on the skin and progressive dyspnea. He was diagnosed as having XD by skin biopsy. [doi.org]
Workup
Progress logically from the histologic pattern, through the appropriate workup, around the pitfalls, to the best diagnosis. [books.google.it]
Systemic examinations including oropharyngeal, ophthalmic, and otorhinolaryngological workup, and laboratory investigations including blood counts, serum biochemistry, thyroid function tests, lipidprofile, urinalysis, ECG, chest X-ray, and ultrasonographic [ijdvl.com]
A thorough workup is essential to identify the underlying condition in order to initiate early treatment and to prevent later complications. [austinpublishinggroup.com]
The macroscopic lesions arise from phagocytosis in the dermis of plasma lipoproteins that leak from capillaries. 1 Laboratory workup is mandatory to exclude diabetes, nephrotic syndrome, or hypothyroidism. [hkmj.org]
Biopsy
- Foam Cell
This is supported by the histopathologic findings of XD in that the foam cells are rare and scalloped macrophages dominant in early stage, but well-developed lesions show a mixture of scalloped cells, foamy cells, and inflammatory cells 3. [ncbi.nlm.nih.gov]
Diffuse histiocytic proliferation, dermal infiltration of lymphocytes, neutrophils, and plasma cells (H and E, ×4), Arrows indicate a (b) touton giant cell and (c) foam cell (H and E, ×40). [ijdvl.com]
Microscopically, these lesions contain an abundant amount of foam cells. Basically, the foam cells are macrophages. Over time, these macrophages have eaten lots of lipid and cholesterol which has accumulated inside the cell. [xanthelasmatreatment.com]
Expected results of diagnostic studies Histopathology Skin biopsy specimens of xanthogranuloma disseminatum will demonstrate a mixture of histiocytes, foam cells, and inflammatory cells. [dermatologyadvisor.com]
Treatment
We studied 15 articles on treatment of xanthoma disseminatum. Local treatment with cryotherapy, radiotherapy, surgery, and carbon dioxide lasers have been attempted with various results. [ncbi.nlm.nih.gov]
Treatment was started with 2-CdA for six 1-month 6 cycles (5 days of treatment in 1 week and repeated every month). [jamanetwork.com]
Prognosis
CNS involvement carries a poor prognosis in this otherwise benign condition. [ncbi.nlm.nih.gov]
Prognosis often resistant to treatment Localization mucocutaneous ocular laryngeal pituitary central nervous system liver ( 16354260 ) See also systemic histiocytoses non-Langerhans cell histiocytoses dermal dendrocytes dendrocytic tumors (dendrocytic [humpath.com]
Even with this escalation of treatment, progressive cases of XD typically have a poor prognosis. [dermatologyadvisor.com]
What is the Prognosis of Xanthoma Disseminatum? (Outcomes/Resolutions) The prognosis of Xanthoma Disseminatum (XD) depends on the type of condition and the symptoms that manifest. [dovemed.com]
Etiology
Xanthoma disseminatum is a rare, benign, non-Langerhans' cell histiocytic disorder of unknown etiology. [ncbi.nlm.nih.gov]
Xanthoma Disseminatum: Case Report Abstract Xanthoma disseminatum is a rare, benign, non-Langerhans´ cell histiocytic disorder of unknown etiology. [adc.mef.hr]
Tumoral pathology > xanthoma disseminatum Xanthoma disseminatum is a rare, usually self- healing dermatologic disease of unknown etiology. Involvement of other organs and tissues including bone marrow, bone, and brain may be seen rarely in children. [humpath.com]
Epidemiology
Definition / general Localized mass forming collection of lipid bearing histiocytes Terminology Also called xanthomatous polyp, xanthogranulomatous inflammation, xanthelasma Epidemiology Very rare in colon - stomach is most common gastrointestinal site [pathologyoutlines.com]
XD could be differentiated from BCH on the basis of epidemiological, clinical and histopathological findings. One hypothesis could link such circumscribed localization to some regional (dis-)immune factors (10). [medicaljournals.se]
Epidemiology Preferentially affects children, usually occurs by 10 years of age, with one-half of reported cases occurring in the first year of life. Twenty percent of the cases occur in adolescents and young adults. [atlasgeneticsoncology.org]
Pathophysiology
Etiology Pathophysiology The etiology and pathogenesis of XD are unknown. Some authors have speculated that the association with paraproteinemia suggests an immunologic basis for this disease. [dermatologyadvisor.com]
Department of Molecular Medicine, IRCCS San Matteo Foundation, University of Pavia, Pavia, 2 Unit of Dermatology, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Via Pace 9, Milan, 3 Department of Oncology and Hematology, and 4 Department of Pathophysiology [medicaljournals.se]
Etiology Pathophysiology Xanthomas are a cutaneous expression of abnormal lipoprotein metabolism, content, or structure, leading to accumulation of lipoproteins in plasma and / or deposition of lipoproteins or their components in tissues. [clinicaladvisor.com]
Prevention
Xanthomas associated with hyperlipidemia require treatment of the lipid disorder to reduce the xanthomas and prevent possible risks (such as atherosclerosis) (2). [empowher.com]
Treatment of eruptive xanthoma involves dietary restriction, exercise, and drug therapy to control the hyperlipidemia and the diabetes. 2 Early recognition and proper control of hypertriglyceridemia can prevent sequelae such as acute pancreatitis. 3 [mdedge.com]
A thorough workup is essential to identify the underlying condition in order to initiate early treatment and to prevent later complications. [austinpublishinggroup.com]
Lipoma disseminatum 1 Department of Medicine, Lady Hardinge Medical College and Smt.S.K.Hospital, New Delhi, India 2 Department of Medicine/Preventive Cardiology, Hamdard Institute of Medical Sciences and Research and HAH Centenary Hospital, Jamia Hamdard [jscisociety.com]