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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 PDFExploring Pediatric Dermatology in Skin of Color: Focus on Dermoscopy.
Life (Basel)
December 2024
Institute of Dermatology, Department of Medical Area, University of Udine, 33100 Udine, Italy.
This literature review aims to comprehensively evaluate the clinical and dermoscopic presentations of common pediatric diseases among children with skin of color (SoC) while also addressing potential variations based on racial backgrounds. This review encompasses various conditions, such as nevi subtypes, viral infections, infestations, and inflammatory dermatoses, as well as hair diseases and abnormal vascular formations, occurring in pediatric populations. Overall, we identified 7 studies on nevi subtypes, 24 studies on skin infections, 6 on inflammatory dermatoses, 10 on hair diseases and disorders, and 14 on miscellaneous disorders that also satisfied our SoC- and race-specific criteria.
View Article and Find Full Text PDFPersonalized Medicine in Histiocytic Disorders: Novel Targets in Patients Without MAPK Alterations.
JCO Precis Oncol
November 2024
Department of Hematology, Mayo Clinic, Rochester, MN.
Purpose: BRAF and MEK inhibitors are standard treatments in histiocytic disorders, such as Erdheim-Chester disease (ECD). Some patients lack MAPK-pathway alterations, making these treatments less effective.
Methods: We describe three patients with histiocytic disorders who have novel non-MAPK pathway alterations.
Disseminated 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.
Multisystem ALK-Positive Histiocytosis With DCTN1::ALK Fusion in an Adult, Responsive to Alectinib: Case Report and Literature Review.
J Cutan Pathol
January 2025
Department of Dermatology, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
Anaplastic lymphoma kinase (ALK)-positive histiocytosis has emerged as a clinically relevant diagnosis featuring a wide span of clinical presentations, which are unified by the presence of ALK-positive histiocytes on histopathology and molecular drivers involving the ALK kinase gene. This report presents an adult case of multisystem ALK-positive histiocytosis with xanthogranuloma-like features on histopathology that was responsive to ALK inhibition, and includes a review of ALK-positive histiocytoses with cutaneous involvement reported in the literature. A 56-year-old male developed a widespread eruption of red-brown papules on the face, trunk, and upper extremities.
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