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Progressive anterior segment involvement of an infant with corneoscleral juvenile xanthogranuloma refractory to topical steroids.
Am J Ophthalmol Case Rep
December 2024
Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Miami, FL, USA.
Purpose: To report a case of corneoscleral juvenile xanthogranuloma (JXG) with progressive anterior segment involvement refractory to topical steroids.
Observations: A 4-month-old male was referred for a new-onset subconjunctival lesion in the right eye. He was found to have a thickened, yellow corneoscleral lesion and hyphema, presumed to be ocular JXG.
Langerhans cell histiocytosis (LCH), juvenile xanthogranuloma (JXG) family lesions, and Rosai-Dorfman-Destombes disease (RDD) are now classified by the World Health Organization (WHO) under the heading of histiocytic/dendritic cell neoplasms. Each disease may manifest as a focal lesion, as multiple lesions, or as a widespread aggressive systemic disease with visceral organ involvement. Erdheim-Chester disease (ECD) is a rare systemic disease process of adults with limited cases in children.
View Article and Find Full Text PDFFoamy Cell Angiosarcoma Resembling a Xanthomatous Histiocytic Infiltrate Without Evidence of Vascular Malignancy.
J Cutan Pathol
December 2024
Department of Medicine, Division of Dermatology, St. Louis School of Medicine, Washington University, St. Louis, Missouri, USA.
Angiosarcoma is a rare and aggressive malignancy of endothelial cells with multiple subtypes. Foamy cell angiosarcoma is a rare variant in which endothelial cells demonstrate "foamy" cytoplasmic change. We present the case of a 59-year-old male who presented with progressive erythema and swelling of the midface and bilateral eyelids.
View Article and Find Full Text PDFDisseminated adult xanthogranuloma in association with underlying B-cell acute lymphoblastic leukemia.
Int J Dermatol
November 2024
Department of Dermatology, University of Maryland School of Medicine, Baltimore, MD, USA.
Generalized Eruptive Histiocytosis or Juvenile Xanthogranuloma: A Clinicopathological Conundrum of Coexistence or Xanthomatization.
Indian Dermatol Online J
July 2024
Department of Dermatology, Venereology and Leprosy, Sri Manakula Vinayagar Medical College and Hospital, Pondicherry, India.
Article Synopsis
- Diseases classified under non-LCH often have similar clinical and histological characteristics, making diagnosis difficult.
- A two-year-old girl exhibited various asymptomatic skin lesions, leading to a provisional diagnosis of generalized eruptive histiocytosis (GEH), which was later found to have overlapping features with juvenile xanthogranuloma (JXG).
- The case highlights the importance of careful differentiation between GEH and JXG since they may require different treatment approaches, demonstrating that clinical observation alone can be misleading due to their similarities.
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