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Xanthoma disseminatum Pathology

Das Med-Tech Startup entwickelt Software für die strukturierte Befundung der Stufe 6 Niedrige Preise, Riesen-Auswahl. Kostenlose Lieferung möglic Xanthoma disseminatum (XD) is a rare disorder that may affect children and adults. Numerous, symmetric reddish yellow or brown papules and nodules are found involving skin folds and on flexor areas of the head and upper body. They are initially discrete, but over time coalesce into verrucous plaques. Lesions may resolve or persist Xanthoma disseminatum. Clinical, metabolic, pathologic, and radiologic aspects. Mishkel MA, Cockshott WP, Nazir DJ, Rosenthal D, Spaulding WP, Wynn-Williams A. A 55-year-old man with the classical mucocutaneous lesions of xanthoma disseminatum has been followed up for a period of 13 years

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Case reports. 38 year old woman with xanthogranulomatous inflammation presenting as a submucosal mass of the sigmoid colon ( Pathol Int 2005;55:440 ) 68 year old man with xanthoma disseminatum with large plaques confined to the back, pulmonary involvement and multiple intestinal xanthomas ( Dermatology 2004;208:164 Introduction: Xanthoma disseminatum (XD) is a rare, benign, non-Langerhans cell histiocytic disorder with unknown etio-pathology. It manifests with multiple, grouped, red-brown to yellow papules and nodules involving the skin, mucous membranes, and internal organs with a predilection for flexures and the face Xanthoma Disseminatum Clinical, Metabolic, Pathologic, and Radiologic Aspects Maurice A. Mishkel, MD; W. Peter Cockshott, MD; Darius J. Nazir, PhD; Donald Rosenthal, MD; William P. Spaulding, MD; Alun Wynn-Williams, MD \s=b\A 55-year-old man with the classical mucocutaneous lesions of xanthoma dis-seminatum has been followed up for a period of 13 years. The special features of this case, which. Xanthoma disseminatum (XD) is a rare, normolipemic form of histiocytosis. Patients rapidly develop hundreds of red-brown papules that become increasingly yellow with time. The lesions are symmetrically distributed and involve the eyelids, trunk, face, and flexural folds of the proximal extremities Xanthoma disseminatum is a normolipemic disseminated xanthomatosis that tends to localize on the flexural and intertriginous surfaces, is often associated with diabetes insipidus, and appears to run a chronic benign course. Xanthomatous deposits may also be found on the mucous membranes of the mouth, pharynx and upper respiratory tract, and on.

Xanthoma is often a clinical diagnosis made in the setting of a dyslipidaemia. Skin biopsy may be required and shows the characteristic lipid-filled macrophages in the dermis (see Eruptive xanthoma pathology). Investigations are required to determine the associated condition and may include Hisanaga Y, Akaike Y, Kuroda K. Xanthoma disseminatum with large plaques confined to the back, pulmonary involvement and multiple intestinal xanthomas. Dermatology . 2004. 208(2):164-6. [Medline]

David Weedon AO MD FRCPA FCAP(HON), in Weedon's Skin Pathology (Third Edition), 2010. Xanthelasma. Xanthelasma is the best known and most common form of xanthoma and is characterized by one or more yellowish plaques on the eyelids or in periorbital skin. 861 In one patient, the xanthelasma was unilateral, sparing a paralyzed lid. 862 Lipid levels are normal in approximately 50% of affected. Xanthoma disseminatum is a rare, benign proliferative disorder characterized by extensive cutaneous and mucous membrane xanthomas in normolipidemic patients. Central nervous system involvement is rare and usually occurs in the systemic variety. Pituitary stalk disease commonly causes hyperprolactine

Diana Bell, in Gnepp's Diagnostic Surgical Pathology of the Head and Neck (Third Edition), 2021. Clinical Features. Xanthogranulomas are nodules in the skin of neonates, infants, and adults that are characterized histologically by large foamy macrophages (xanthoma cells). Their natural history and age distribution suggest that they are not neoplasms but reactive developmental/metabolic or. Xanthoma disseminatum. Gong HZ (1), Zheng HY (1), Li J (2). Author information: (1)Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China

1. J R Soc Med. 1985;78 Suppl 11:8-9. Xanthoma disseminatum. Ford GP, Eady RD, Freemont AJ. PMCID: PMC1289451 PMID: 3841561 [PubMed - indexed for MEDLINE Xanthoma disseminatum is a non-Langerhans histiocytic proliferation first described by Montgomery in 1938. This rare entity is characterized by skin and mucous membrane xanthomatosis in which the facial involvement is common, together with diabetes insipidus and normal lipid metabolism

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Purpose: Xanthoma disseminatum is a rare non-Langerhans' histiocytosis of older children and adults with characteristic lesions involving the skin, mucous membrane and occasionally internal organs. We describe a case, presenting in infancy, with unique clinical features Xanthoma disseminatum (XD) is a rare benign histiocytic proliferating disease of non-Langerhans cell origin [ 1, 2 ]. It is clinically mainly characterized by a number of symmetrically distributed cutaneous yellow-brown papules, often affecting faces, trunk and limbs Gustave L. Davis, in Diagnostic Surgical Pathology of the Head and Neck (Second Edition), 2009 Clinical Features. Xanthogranulomas are nodules in the skin of neonates, infants, and adults that are characterized histologically by large foamy macrophages (xanthoma cells). Their natural history and age distribution suggest that they are not neoplasms but reactive developmental/ metabolic or. Histological examination of resected xanthomas revealed dense infiltrations of the interstitial spaces by foam-cell histiocytes with multiple lipid vacuoles, typical of xanthoma disseminatum. Treatment and course: Neither probucol nor cholesterol synthesis enzyme inhibitors nor glucocorticoid medication influenced the xanthomas • Background and Design.— As xanthoma disseminatum and histiocytosis X share clinicopathologic features, difficulties in diagnosis can arise. The use of immunocytochemical markers for S100 protein, factor XIIIa, lysozyme, α1-antitrypsin, for adherence of peanut agglutinin and of antibodies LN3 (HLA-DR), Leu-M1 (CD15), QBEnd/10 (CD34), MAC 387, and KP1 (CD68) as an aid to conventional.

Xanthoma disseminatum - an overview ScienceDirect Topic

Disseminated xanthosiderohistiocytosis, a variant of xanthoma disseminatum, in a patient with a plasma cell dyscrasia.  J Am Acad Dermatol . 1984;11(4 pt 2):750-755. doi: 10.1016/S0190-9622(84)70236-2 PubMed Google Scholar Crossre Introduction: Xanthoma disseminatum is a very rare disease classified as a benign non-Langerhans cell histiocytosis, which is rarely associated with osteoarticular lesions. There is only a report of tumor abrasion during treatment of osteoarticular lesions of this disease, artificial joint replacement has not been reported Keywords: Xanthoma disseminatum, Disseminated, Histiocytic neoplasm, MR, Immunohistochemistry Background Xanthoma disseminatum (XD) is a rare benign histiocytic proliferating disease of non-Langerhans cell origin [1, 2]. It is clinically mainly characterized by a number of symmet-rically distributed cutaneou s yellow-brown papules, ofte The hallmark histopathologic feature of most xanthomas is the occurrence of foam skin cells within the dermis. These skin cells represent macrophages that have accumulated lipid. These skin cells will stain positive for lipid with special staining (Oil-red-O)

Xanthoma disseminatum

Xanthoma Disseminatum However, this disease is not due to the xanthoma dissemination, but due to the underlying pathology. Xanthoma dissemination can also affect the internal organs including lungs, liver, and kidneys, complicating the clinical presentation. The disease usually occurs in boys and in young age and is self-limiting Xanthoma disseminatum (XD) is a rare benign mucocutaneous xanthomatosis that is classified as a benign non-Langerhans cell histiocytosis. We report a 62-year-old man who presented with widespread yellow-brown papulonodular and tumoral lesions on face, flexors, and trunk. Histopathological features of the cutaneous lesions were typical of XD Xanthoma disseminatum with large plaques confined to the back, pulmonary involvement and multiple intestinal xanthomas Dermatology , 208 ( 2 ) ( 2004 ) , pp. 164 - 166 View Record in Scopus Google Schola The Xanthogranulomatous Process (XP), is a form of acute and chronic inflammation characterized by an exuberant clustering of foamy macrophages among other inflammatory cells

Xanthoma Disseminatum of the Central Nervous System and

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Punch biopsy revealed numerous histiocyte infiltrates with large vacuoles, xanthomized cytoplasm in the dermis, and midsection, and there are several Touton-type giant cells supporting the preliminary diagnosis of xanthoma disseminatum Xanthoma disseminatum (XD) is a rare non-Langerhans cell histiocytosis that is characterized by the proliferation of histiocytic cells and secondary deposition of lipids in the dermis, which can affect people at any age. Men are more commonly affected than women. 1 Treating XD is challenging. We present a patient with XD with marked facial. Clinics and Pathology Phenotype / cell stem origin Rudolf Virchow may have been the first to describe a child with cutaneous xanthomas in 1871 as noted in a 1954 report of this condition (Helwig and Hackney, 1954) Introduction: Xanthoma disseminatum (XD) is a rare, benign, non-Langerhans cell histiocytic disorder with unknown etio-pathology. It manifests with multiple, grouped, red-brown to yellow papules and nodules involving the skin, mucous membranes, and internal organs with a predilection for flexures and the face. Case Report: We report a patient who presented with disseminated xanthomatous. Xanthoma disseminatum consists of numerous, small, reddish-brown papules that are evenly spread over the face, skin-folds, trunk, and proximal extremities. Juvenile xanthogranuloma occurs primarily in children and is characterized by discrete yellowish-orange nodules, which commonly appear on the scalp, face, and upper trunk

Xanthoma Disseminatum Presenting with Hoarseness

Xanthoma Disseminatum. Xanthoma-like lesions anticipated to an uncommon form of histiocytosis. Lipid metabolism is normal. The skin lesions are a huge selection of small yellowish-brown or reddish-brown bumps, which can be cover the facial skin and trunk Xanthoma disseminatum presents with multiple papules and nodules, symmetrically distributed over the trunk, face, and extremities. These lesions become confluent to form plaques. Mucous membranes are involved in 30% of cases. Meningeal involvement is common and occur in the form of diabetes insipidus (40% of cases), seizures and growth retardation It was first described in 3 cases in 1998 (Folkerth, RD Am J Surg Pathol 22: 736-41). Since then only 4 additional cases have appeared in the literature: one in Cheung et al (JCEM 86: 1048-53, 2001), one in Deodhare, SS et al (Endocrine Pathology 10: 237-241, 1999), and two in Tashiro et al (Endocrine Pathology 13: 183-195, 2002) Xanthoma disseminatum (XD) is a rare, benign, non‐Langerhans cell histiocytosis characterized by disseminated xanthomatous lesions with a predilection for the face, flexures, and mucosae. Approximately 100 cases have been reported in the literature Pediatric Dermatology 1985;3:19-30. 22. Kumakiri M, et al. Xanthoma disseminatum: report of a case 8. Caputo R, et al. The various clinical patterns of xanthoma with histologic and ultrastructural studies of skin lesions. disseminatum: consideration of 7 cases and review of the J Am Acad Dermatol 1983;4:291-9. literature

Zak IT, Altinok D, Neilsen SS, Kish KK. Xanthoma disseminatum of the central nervous system and cranium. AJNR Am J Neuroradiol. 2006 Apr. 27(4):919-21. . Gupta P, Khandpur S, Vedi K, Singh MK, Walia R. Xanthoma disseminatum associated with inflammatory arthritis and synovitis--a rare association. Pediatr Dermatol. 2015 Jan-Feb. 32(1):e1-4 Xanthoma disseminatum (XD) is a rare mucocutaneous xanthomatosis classified as a benign form of non-Langerhans' cell histiocytosis. The case history is presented of a 61 year old woman with XD who developed dyspnoea and spirometric features of airflow obstruction. Bronchoscopy and computed tomography confirmed involvement of the large and medium sized bronchi and she subsequently died from. Xanthoma disseminatum, also part of the non-Langerhans histiocytic disorder spectrum, typically occurs in teenagers and young adults. The skin lesions are described as yellow to brown growths forming plaques and nodules that can present anywhere on the skin but most commonly in flexural areas of the extremities, the eyelids, and mucosae.

Training And Research Hospital, Pathology, Istanbul, Turkey (2) Introduction: Xanthoma disseminatum (XD) is a rare xanthogranulomatous disease with unknown etiology which is classified into non-Langerhans cell histiocytosis. Dermoscopic features of the lesion had been reported. As far as we know, confocal microscopic feature Xanthoma disseminatum is a rare, benign proliferative disorder characterized by extensive cutaneous and mucous membrane xanthomas in normolipidemic patients. Central nervous system involvement is rare and usually occurs in the systemic variety

Xanthoma disseminatum (xanthogranuloma disseminatum

  1. atum (XD) is an extremely rare cutaneous, normolipidemic, proliferative disorder of the Mononuclear Phagocyte System. It was first described by Von Gräfe and Virchow but recognized as a distinct entity by Montgomery & Osterberg in 1938 (1)
  2. atum (XD) is a rare normolipemic mucocutaneous xanthomatosis due to the proliferation of non-x histiocytes. Occasional involvement of the upper respiratory system has been reported, but lower respiratory tract involvement is very rare
  3. atum: a rare xanthoma consisting of non-X histiocytes on flexural (folded) surfaces, associated with diabetes insipidus. Verrucous xanthoma, or histiocytosis Y: a papilloma of the oral mucosa and skin whereby the connective tissue under the epithelium contains histiocytes

• Xanthoma disseminatum • Ceroid granuloma of the gallbladder, • Whipple's disease • Inflammatory pseudotumor of the lung • Plasma cell granuloma of the lung • Malakoplakia • Verruciform xanthoma • Foamy histiocytosis of the spleen in thrombocytopenic purpura • Isolated xanthoma of the small bowel • Xanthofibroma of bon Ito S, et al. Colorectal xanthomas with polypoid lesion: report of 25 cases. Acta Pathol Microbiol Immunol Scand 2004;112(1): 3-10. [Review]. [11] Hisanaga Y, Akaike Y, Kuroda K. Xanthoma disseminatum with large plaques confined to the back, pulmonary involvement and multiple intestinal xanthomas. Dermatology 2004;208(2): 164-6 Noncutaneous xanthomas might be more problematic to diagnose. In the case of a laryngeal xanthoma, the differential diagnosis includes several processes, including xanthoma disseminatum affecting the larynx , which we ruled out clinically because of the absence of other xanthomas in the skin. Histologically, we also excluded the diagnoses of. 22 Kumakiri M, et al. Xanthoma disseminatum: report of a case with histologic and ultrastructural studies of skin lesions. J Am Acad Dermatol 1983;4:291-9. Crossref ISI, Google Scholar. 23 Blobstein SH, Caldwell D, Carter M. Bone lesions in xanthoma disseminatum. Arch Dermatol 1985;121:1313-7. Crossref Medline, Google Schola

Xanthoma disseminatum (XD) is a rare, benign, non-Langerhans cell histiocytic disorder. The pathogenesis is not clear. It manifests with multiple, grouped, red-brown to yellow papules and nodules involving the skin, mucous membranes, and internal organs Topics: diagnostic use: Antibodies, Monoclonal, Axilla, Female, pathology: Histiocytes, Humans, Middle Aged, pathology: Skin, diagnosis: Skin Diseases, diagnosis. Generalized eruptive histiocytoma (GEH) is a non-Langherhans cell histiocytosis (Table 1). These disorders share the same immunophenotypes but differ in clinical presentation, population affected, and disease course and prognosis. Characteristic findings on physical examination. Adults present most commonly in the fourth to sixth decade and. It is not uncommon to find xanthoma disseminatum but rare to find lipoma disseminatum. Acknowledgement We are thankful to Dr. Sabina Khan and Dr. Zeeba S. Jairajpuri, Asssitant Professor, Department of Pathology, HIMSR for their contributions in tissue diagnosis BACKGROUND: Xanthoma disseminatum is a rare nonfamilial disease characterized by lipid deposition in skin and internal organs due to histiocytic cell proliferation, classified as a benign non-Langerhans cell histiocytosis. This chronic disease has no known established treatment. We report 8 cases of.

Xanthoma Disseminatum JAMA Dermatology JAMA Networ

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Verruciform xanthoma: A view on the concepts of its etiopathogenesis. Journal of oral and maxillofacial pathology: JOMFP. 2013;17(3):392. 9. Park M, Boone B, Devos S. Xanthoma disseminatum: case report and mini-review of the literature. Actadermato venerologica Croatica. 2014;22(2):150-5. 10 Hisanaga Y, Akaike Y, Kuroda K: Xanthoma disseminatum with large plaques confined to the back, pulmonary involvement and multiple intestinal xanthomas. Dermatology. 2004, 208:164-166. 10.1159/000076494; Miliauskas JR: Rectosigmoid (colonic) xanthoma: a report of four cases and review of the literature. Pathology. 2002, 34:144-147 Two cases of rectal xanthomas are described. One case is that of a 56-year-old Japanese man in whom multiple yellowish spots measuring approximately 3 to 5 mm were observed in the rectum during colonoscopy. The other case is that of a 78-year-old Japanese man in whom colonoscopy showed a whitish plaque of 4 mm in diameter in the rectum

Juvenile xanthogranuloma is a type of non-Langerhan's cell histiocytosis (Class IIb). It presents as skin lesions predominantly in infants and young children, more often males, and is present at birth in 20% of cases. However, 10% of cases are adults Factor XIIIa is a blood coagulation prototransglutaminase 1, which becomes an activated component of the final stages of the clotting cascade as a result of the interaction with thrombin and Ca 2+.FXIIIa plays a key role in fibrin stabilization, and defective fibrin stabilization is demonstrable as a result of severe haemorrhagic manifestations of congenital FXIII deficiency, which is an. Xanthoma disseminatum (XD) is composed of Factor XIIIa-positive histiocytes and may give a clinical presentation similar to ECD in that XD patients often have periorbital xanthomas and.

Letters 62 to the Editors Hypopituitarism and rare dermatological diseases: an intriguing case of xanthoma disseminatum Hypopituitarism is a rare condition caused by granulomatous dis- Fig. 1 Skin manifestations of xanthoma disseminatum: the black arrows eases in about 1% of cases.1 Among proliferative granulomatous show multiple red-yellowish. The various clinical patterns of xanthoma disseminatum. Considerations on seven cases and review of the literature. Dermatology 1995; 190:19. Park M, Boone B, Devos S. Xanthoma disseminatum: case report and mini-review of the literature. Acta Dermatovenerol Croat 2014; 22:150. Gong HZ, Zheng HY, Li J. Xanthoma disseminatum. Lancet 2018; 391:251 xanthelasma, xanthoma disseminatum, plane xanthomas, eruptive xanthomas [7]. Xanthomas develop as a swollen, thickened yellow coloured skin in the birds [13]. Grossly, the xanthomas appear yellow to white in color which could be attributed to their lipid content. It could be variably sized. Characteristic histopathological features are indicated b Xanthoma disseminatum. Malignant histiocytic disorders. Surgical pathology associated with epilepsy. Focal cortical dysplasia. Cortical tubers. Hemimegalencephaly. Polymicrogyria. Cerebral heterotopias. Perivascular oligodendrocyte clustering/hyperplasia. Classical hippocampal sclerosis The pathology consists of perivascular demyelination, as well as hemorrhage and necrosis of vessels. References: Adams RD, Cammermeyer J, Denny-Brown D. Acute necrotizing hemorrhagic encephalopathy. J Neuropathol Exp Neuro11949; 8:1-29. Case Records of the Massachusetts General Hospital

Histiocytoses are rare diseases caused by an excess of cells called Histiocytes, which can infiltrate the skin, bones, lungs, liver, spleen and the central nervous system. These disorders can range from localized involvement that resolves spontaneously, to progressive disseminated forms that can be debilitating and sometimes life-threatening INTRODUCTION. Xanthomas are localized lipid deposits within organs that may manifest as papules, plaques, or nodules in skin. The clinical variants of cutaneous xanthomas include: Eruptive xanthomas ( picture 1A-C) Tuberous xanthomas ( picture 2A-C) Tendinous xanthomas ( picture 3 Xanthoma disseminatum involving the hypophyseal stalk had a pyramidal pattern of enhancement on MRI. This rare histiocytosis syndrome is characterized by disseminated xanthomatous lesions that involve the skin, particularly the flexor folds and eyelids; the central nervous system, respiratory tract, and gastrointestinal tract may also be.

What is the pathophysiology of xanthomas

Indeterminate cell histiocytosis (ICH) is a rare disorder involving altered homing mechanisms of the cutaneous histiocytic/dendritic system. Indeterminate cells belong to the epidermal/dermal dendritic cell system and reside in the skin and mucosa; they resemble Langerhans cells morphologically, cytochemically, and phenotypically but, unlike them, lack Birbeck granules.1 ICH has been described. The patient suffered from diplopia and had a severe sensorimotor polyneuropathy causing progressive paresis of the limbs. The explorations performed disclosed the presence of specific xanthomatous infiltrates in the skin, lungs, respiratory tract, peripheral nerves and meninges, suggesting xanthoma disseminatum (XD) or juvenile xanthogranuloma Xanthoma disseminatum is a rare, predominantly cutaneous non-Langerhans cell histiocytosis. Xanthoma disseminatum is so named for its characteristic skin lesions. However, this is a misnomer, as it is a disorder of histiocytes rather than lipid metabolism as in true xanthomata Xanthoma disseminatum and verruciform xanthoma are particular forms of xanthomas that occur in normolipemic patients. [68, 69] Xanthoma disseminatum presents in adults most commonly as red-yellow papules and nodules with a predilection for the flexures. They may unusually have extensive eyelid involvement. [34

Methotrexate-induced papular eruption in patients withHEMANTH VAMANSHANKAR | MBBS, DLO, DNB-ENT, DOHNS (EdinHistopathology and selective biomarker expression in human

Define xanthomas. xanthomas synonyms, xanthomas pronunciation, xanthomas translation, English dictionary definition of xanthomas. (Pathology) pathol the presence in the skin of fatty yellow or brownish plaques or nodules, xanthoma disseminatum - rare chronic xanthoma of adults in which orange or brownish papules develop on many surfaces. Xanthoma disseminatum is a self-limiting disease but may persist for years. Skin lesions of xanthoma disseminatum are disfiguring and patients often request treatment. The carbon dioxide laser has been used with good results.6 (including upper eyelids), peri Azathioprine and cyclophosphamide have been effectiv Xanthoma disseminatum of the central nervous system and cranium. Zak IT , Altinok D , Neilsen SS , Kish KK AJNR Am J Neuroradiol , 27(4):919-921, 01 Apr 200 Thus, the diagnosis is non-LCH with joints and lymph nodes involvement. This patient presented both the clinical manifestations of multicentric reticulohistiocytosis (MRH) and histologic findings of xanthoma disseminatum (XD), indicating the plausible association and the shared disease spectrum of MRH and XD (Pathology) pathol the presence in the skin of fatty yellow or brownish plaques or nodules, esp on the eyelids, xanthoma disseminatum - rare chronic xanthoma of adults in which orange or brownish papules develop on many surfaces of the body. cholesterosis cutis,.