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Numerous asymptomatic papulo-nodules and plaques in a young male.

Das D, Das A, Saha A, Sardar S, Shome K, Gharami RC - Indian J Dermatol (2015 Jan-Feb)

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology, Medical College and Hospital, Kolkata, West Bengal, India.

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Family history was non-contributory... Systemic examinations including ophthalmologic, oropharyngeal and otorhinolaryngological referral were done and nothing was found to be abnormal... Histopathological examination in all the specimens showed diffuse proliferation of histiocytes, admixed with Touton giant cells, foam cells, scalloped macrophages, lymphocytes and eosinophils in the dermis [Figures 4–6]... Xanthoma disseminatum (XD) is a condition in which lipid deposition occurs secondary to a histiocytic proliferation... This histiocytic proliferation goes in favor of a histiocytic disorder, with Touton giant cells representing an accentuated xanthomatoid reaction, and immunohistochemistry staining positive for CD 68 and factor XIIIa support it being a disorder of histiocytes/macrophages, whereas negative staining for S-100, CD1a and Birbeck granules reliably excludes Langerhans cell histiocytosis... In early lesions, scalloped macrophages dominate the histologic picture... A table showing the salient points of difference between papular xanthoma, progressive nodular histiocytosis, generalized eruptive histiocytoma and XD has been provided [Table 1]... The treatment modalities have shown variable results... Eisendle et al. reported that oral prednisolone and azathioprine showed no improvement but a combination of lipid-lowering agents or azathioprine and cyclophosphamide was found to be useful... Kang et al. reported the successful use of a combination of oral steroids, clofibrate, and chemotherapy... Bone marrow transplantation has been used successfully in a case of life-threatening xanthogranuloma disseminatum in neurofibromatosis type-1 in a recent report by Savaşan et al... In 2011, Khezri et al. reported a case series in which 2-chlorodeoxyadenosine therapy was found useful in maintaining remission and long-term control of cutaneous lesions, wherein among eight cases of XD, a positive response to treatment with 2-chlorodeoxyadenosine was seen in five cases.

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Photomicrograph showing a dermal lesion composed of sheets of scalloped macrophages (arrow) and histiocytes (H and E, ×40)
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Figure 5: Photomicrograph showing a dermal lesion composed of sheets of scalloped macrophages (arrow) and histiocytes (H and E, ×40)


Numerous asymptomatic papulo-nodules and plaques in a young male.

Das D, Das A, Saha A, Sardar S, Shome K, Gharami RC - Indian J Dermatol (2015 Jan-Feb)

Photomicrograph showing a dermal lesion composed of sheets of scalloped macrophages (arrow) and histiocytes (H and E, ×40)
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4318079&req=5

Figure 5: Photomicrograph showing a dermal lesion composed of sheets of scalloped macrophages (arrow) and histiocytes (H and E, ×40)

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology, Medical College and Hospital, Kolkata, West Bengal, India.

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

Family history was non-contributory... Systemic examinations including ophthalmologic, oropharyngeal and otorhinolaryngological referral were done and nothing was found to be abnormal... Histopathological examination in all the specimens showed diffuse proliferation of histiocytes, admixed with Touton giant cells, foam cells, scalloped macrophages, lymphocytes and eosinophils in the dermis [Figures 4–6]... Xanthoma disseminatum (XD) is a condition in which lipid deposition occurs secondary to a histiocytic proliferation... This histiocytic proliferation goes in favor of a histiocytic disorder, with Touton giant cells representing an accentuated xanthomatoid reaction, and immunohistochemistry staining positive for CD 68 and factor XIIIa support it being a disorder of histiocytes/macrophages, whereas negative staining for S-100, CD1a and Birbeck granules reliably excludes Langerhans cell histiocytosis... In early lesions, scalloped macrophages dominate the histologic picture... A table showing the salient points of difference between papular xanthoma, progressive nodular histiocytosis, generalized eruptive histiocytoma and XD has been provided [Table 1]... The treatment modalities have shown variable results... Eisendle et al. reported that oral prednisolone and azathioprine showed no improvement but a combination of lipid-lowering agents or azathioprine and cyclophosphamide was found to be useful... Kang et al. reported the successful use of a combination of oral steroids, clofibrate, and chemotherapy... Bone marrow transplantation has been used successfully in a case of life-threatening xanthogranuloma disseminatum in neurofibromatosis type-1 in a recent report by Savaşan et al... In 2011, Khezri et al. reported a case series in which 2-chlorodeoxyadenosine therapy was found useful in maintaining remission and long-term control of cutaneous lesions, wherein among eight cases of XD, a positive response to treatment with 2-chlorodeoxyadenosine was seen in five cases.

No MeSH data available.


Related in: MedlinePlus