scholarly journals A Rare Case of Erdheim-Chester Disease and Langerhans Cell Histiocytosis Overlap Syndrome

2015 ◽  
Vol 2015 ◽  
pp. 1-7
Author(s):  
Shahzaib Nabi ◽  
Adeel Arshad ◽  
Tarun Jain ◽  
Fawad Virk ◽  
Rohit Gulati ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Retroperitoneal Fibrosis ◽  
Obstructive Uropathy ◽  
Altered Mental Status ◽  
Langerhans Cell ◽  
Erdheim Chester Disease ◽  
Stage Renal Disease ◽  
End Stage ◽  
Erdheim Chester ◽  
Chester Disease

A 48-year-old woman with a past medical history of seizures and end-stage renal disease secondary to obstructive uropathy from retroperitoneal fibrosis presented to the emergency department with seizures and altered mental status. A Glasgow Coma Scale of 4 prompted intubation, and she was subsequently admitted to the intensive care unit. Magnetic resonance imaging of the brain performed to elucidate the aetiology of her seizure showed a dural-based mass within the left temporoparietal lobe as well as mass lesions within the orbits. Further imaging showed extensive retroperitoneal fibrosis extending to the mediastinum with involvement of aorta and posterior pleural space. Imaging of the long bones showed bilateral sclerosis and cortical thickening of the diaphyses. Imaging of the maxillofacial structures showed osseous destructive lesions involving the mandible. These clinical and radiological features were consistent with a diagnosis of Erdheim-Chester disease; however, the patient’s skin biopsy was consistent with Langerhans cell histiocytosis.

Erdheim?Chester disease of the breast associated with Langerhans-cell histiocytosis of the hard palate

2004 ◽  
Vol 445 (4) ◽  
pp. 405-409 ◽  
Author(s):  
V. P. Andrade ◽  
C. C. V. Nemer ◽  
A. N. L. Prezotti ◽  
W. S. L. Goulart
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Hard Palate ◽  
Langerhans Cell ◽  
Erdheim Chester Disease ◽  
Erdheim Chester ◽  
Chester Disease

scholarly journals Dramatic efficacy of dabrafenib in Erdheim-Chester disease (ECD): a pediatric patient with multiple large intracranial ECD lesions hidden by refractory Langerhans cell histiocytosis

2019 ◽  
Vol 23 (1) ◽  
pp. 48-53 ◽  
Author(s):  
Xiaolei Hao ◽  
Ruie Feng ◽  
Yalan Bi ◽  
Yuhan Liu ◽  
Chunde Li ◽  
...  
Keyword(s):  
Pediatric Patient ◽  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Neurological Involvement ◽  
Treatment Processes ◽  
Erdheim Chester Disease ◽  
Intracranial Lesions ◽  
The Central Nervous System ◽  
Erdheim Chester ◽  
Chester Disease

Erdheim-Chester disease (ECD) is a rare non–Langerhans cell form of histiocytosis that can affect the central nervous system. ECD predominantly affects adults, and only a few pediatric cases have been reported. The co-occurrence of ECD and Langerhans cell histiocytosis (LCH) is exceedingly rare. An 11-year-old boy, who was diagnosed with LCH 7 years previously, presented with multiple giant intracranial lesions. At the time of his initial diagnosis, only one intracranial lesion was observed, and it began to enlarge. Currently, up to 7 intracranial lesions can be observed in this patient. However, the diagnosis of ECD was not confirmed until this most recent open resection. The BRAF V600E mutation was detected in both LCH and ECD lesions. Dabrafenib therapy exhibited dramatic efficacy in this pediatric patient. This case represents the first successful application of dabrafenib in a pediatric patient with intracranial ECD lesions as well as mixed ECD and LCH. In this article, the authors describe the intricate diagnosis and treatment processes in this patient. Recent studies regarding treatment with BRAF inhibitors for neurological involvement in mixed ECD and LCH are also reviewed.

Mixed Langerhans Cell Histiocytosis and Erdheim-Chester Disease in a Girl

2020 ◽  
Vol Publish Ahead of Print ◽  
Author(s):  
Suheyla Ocak ◽  
Zuhal Bayramoglu ◽  
Deniz Tugcu ◽  
Serap Karaman ◽  
Aysegul Unuvar ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Erdheim Chester Disease ◽  
Erdheim Chester ◽  
Chester Disease

18F-Fluoride PET/CT Aspect of an Unusual Case of Erdheim-Chester Disease With Histologic Features of Langerhans Cell Histiocytosis

2013 ◽  
Vol 38 (7) ◽  
pp. 541-542 ◽  
Author(s):  
Cécile Caoduro ◽  
Constantin Marius Ungureanu ◽  
Boris Rudenko ◽  
Orland Angoue ◽  
Oleg Blagosklonov ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Unusual Case ◽  
Langerhans Cell ◽  
Erdheim Chester Disease ◽  
Pet Ct ◽  
Erdheim Chester ◽  
18F Fluoride ◽  
Chester Disease

Erdheim—Chester Disease

2003 ◽  
Vol 7 (2) ◽  
pp. 129-132
Author(s):  
Susan E. Lenahan ◽  
Klaus F. Helm ◽  
Kenneth D. Hopper
Keyword(s):  
Differential Diagnosis ◽  
Case Report ◽  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Morbidity And Mortality ◽  
Significant Morbidity ◽  
Erdheim Chester Disease ◽  
Pathologic Findings ◽  
Erdheim Chester ◽  
Chester Disease

Background: Erdheim–Chester disease is a rare non-Langerhans' cell histiocytosis. Objective: This case report is presented to familiarize clinicians with Erdheim–Chester disease and its differential diagnosis. Results and Conclusion: Erdheim–Chester disease presents with unique clinical and pathologic findings. Its xanthoma-like lesions can cause significant morbidity and mortality.

Sign in / Sign up

Export Citation Format

Share Document