Eosinophilic granuloma (EG) is the most common and benign form of the spectrum of disorders referred to as Langerhans cell histiocytosis (LCH). Langerhans cell histiocytosis is primarily regarded as a pediatric disease, with few adult cases of multifocal EG of bone reported. We report a case of multifocal EG in a 48-year-old woman, who presented with right knee pain. Radiographs showed a small lytic lesion in the medial femoral condyle. Diagnosis was confirmed by ultrasound-guided biopsy. She had had a previous EG lesion excised from her skull. Whole-body bone scan demonstrated a new skull lesion in the right diploic space, which was confirmed by magnetic resonance imaging. The patient underwent curettage, bone grafting, and prophylactic internal fixation of the right distal femur lesion. The skull lesion was treated with repeat craniectomy. Two years later, she developed a new lesion in the right distal femoral metaphysis, which was treated with intralesional corticosteroid injections. Now, more than 1 year later, the patient is pain-free with no evidence of new or recurrent disease. Because multifocal EG is a rare diagnosis in adults, appropriate clinical suspicion, in combination with radiographic findings and histologic examination, is essential for correct diagnosis and treatment.
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Haematologica
January 2025
Sorbonne University, Internal Medicine Department 2, Institut E3M, French Reference Centre for Histiocytosis, Pitié-Salp trière.
Not available.
View Article and Find Full Text PDFRheumatology (Oxford)
January 2025
Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele Hospital, Vita-Salute San Raffaele University, Milan, Italy.
Objective: Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis that exhibits a wide spectrum of clinical manifestations. The recent identification of activating mutations in the MAPK-ERK pathway in patients with ECD has led to the introduction of targeted therapies. The most commonly used targeted therapies are BRAF- and MEK-inhibitors, which are highly effective but also carry significant toxicity.
View Article and Find Full Text PDFActa Physiol (Oxf)
February 2025
Department of Medical Cell Biology, Uppsala University, Uppsala, Sweden.
Aim: Somatostatin from pancreatic δ-cells is a paracrine regulator of insulin and glucagon secretion, but the release kinetics and whether secretion is altered in diabetes is unclear. This study aimed to improve understanding of somatostatin secretion by developing a tool for real-time detection of somatostatin release from individual pancreatic islets.
Methods: Reporter cells responding to somatostatin with cytoplasmic Ca concentration ([Ca]) changes were generated by co-expressing somatostatin receptor SSTR2, the G-protein Gα15 and a fluorescent Ca sensor in HeLa cells.
JID Innov
January 2025
Center for Cancer Immunology is a part of Krantz Family Center for Cancer Research, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts, USA.
Adult human skin contains nearly twice as many T cells as the peripheral blood, which include tissue-resident memory T cells. However, the precise mechanisms maintaining tissue-resident memory T cells in the healthy skin remain unclear. Using normal human skin samples, we find that Langerhans cells (LCs) contact T cells in the epidermis of the elderly.
View Article and Find Full Text PDFLangerhans 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.
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