Bielach – Bazyluk, Angelika Serwin, Agnieszka B. Pilaszewicz – Puza, Agata Flisiak, Iwona
Published in
BMC Dermatology
BackgroundCutaneous Rosai – Dorfman disease (CRDD) is extremely rare variant of idiopathic histiocytic proliferative disorder, which may manifest as a non-specific macules, papules, plaques or nodules ranging in size and colour from yellow – red to red -brown.Case presentationA 52-year-old female presented with three gradually enlarging, reddish - ...
Schwartz, Ziv Bender, Anna Magro, Cynthia M
Published in
Pediatric dermatology
Langerhans cell histiocytosis (LCH) is the neoplastic proliferation of dendritic langerin-positive histiocytes manifesting as either single system unifocal, single system multifocal, or multisystem disease. The designation Hashimoto-Pritzker, or self-healing LCH, has fallen out of favor since it is impossible to predict at time of diagnosis whether...
Chan, Warren H Shah, Aatman Bae, Gordon Hambro, Caely Martin, Beth A Brown, Ryanne Novoa, Roberto Kwong, Bernice Y
Published in
JAAD case reports
Lourenço, Jorge Ferreira, Cristina Marado, Daniela
Published in
Molecular and Clinical Oncology
Langerhans cell histiocytosis (LCH) is a rare systemic and heterogeneous disease secondary to proliferation and diffuse infiltration of immature CD1a-positive dendritic cells, also known as Langerhans cells. LCH affects predominantly paediatric patients and is rarely diagnosed in adulthood. Despite its worldwide prevalence, most reported cases are ...
Razanamahery, Jerome Humbert, Sebastien Weil-Verhoeven, Delphine Emile, Jean-Francois Vuitton, Lucine Magy-Bertrand, Nadine
Published in
Inflammatory bowel diseases
Pigem, Ramon Dyer, Ariann Podlipnik, Sebastian Carrera, Cristina Puig, Susana Ferrando, Juan
Published in
Dermatology Practical & Conceptual
Li, Hua Ells, Peter Arslan, Mustafa Erdem Robstad, Karl A Lee, Hwajeong
Published in
Cureus
Langerhans cell histiocytosis (LCH) is a rare systemic disorder characterized by an infiltration of CD1a+/langerin+ histiocytes, commonly involving bone, skin, and lymph nodes in children. Hepatic involvement is rarely observed in multisystem LCH. We describe an exceptional case of hepatic LCH in an adult preceding the diagnosis of multisystem LCH,...
Cyr, Janelle Langley, Annie Demellawy, Dina El Ramien, Michele
Published in
SAGE Open Medical Case Reports
In our case report, we discuss a 1-day-old boy presenting with blueberry muffin syndrome diagnosed with Langerhans cell histiocytosis. The diagnosis complicated by an initial difficult-to-interpret biopsy showing only a hint of perifollicular CD1a-positive cells; however, given our team’s strong clinical suspicion of Langerhans cell histiocytosis, ...
Mazor, Roei D Weissman, Ran Luckman, Judith Domachevsky, Liran Diamond, Eli L Abdel-Wahab, Omar Shapira, Shirley Hershkovitz-Rokah, Oshrat Groshar, David Shpilberg, Ofer
...
Published in
Neuro-oncology Advances
Background Erdheim–Chester disease (ECD), a rare inflammatory myeloid neoplasm, is known to be fundamentally reliant on the constitutive activation of the MAPK signaling pathway in the majority of patients. Consequently, inhibition of the V600E-mutant BRAF kinase has proven to be a safe and efficacious long-term therapeutic strategy for BRAF -mutan...
Sacconi, Riccardo Campochiaro, Corrado Rabiolo, Alessandro Marchese, Alessandro Tomelleri, Alessandro Tomasso, Livia Cicinelli, Maria Vittoria Querques, Lea Bandello, Francesco Dagna, Lorenzo
...
Published in
Clinical Ophthalmology (Auckland, N.Z.)
Purpose To analyze the subclinical intraocular involvement using multimodal imaging approach in patients affected by Erdheim-Chester disease (ECD) without ocular symptoms. Patients and Methods In this prospective cross-sectional study, 18 eyes of 9 consecutive patients with ECD were enrolled. Each patient underwent comprehensive ocular examination ...
