Hourglass fascicular constrictions have been reported in fewer than 100 cases globally and only in the upper limb. The etiology remains unknown. Patients often present with self-limiting pain in the affected limb followed by flaccid paralysis. As the external architecture of the nerve remains normal on traditional radiological studies, the diagnosis can be missed. The purpose of this case report is to describe this rare clinical entity and a successful treatment approach including ultrasound localization and targeted intraneural microsurgical neurolysis followed by excellent functional recovery.
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http://dx.doi.org/10.1097/SAP.0000000000004223 | DOI Listing |
J Low Genit Tract Dis
January 2025
Saint Louis University School of Medicine, St. Louis, MO.
Objective: Authors characterized all published adult cases of cutaneous, intertriginous Langerhans cell histiocytosis (LCH) to bring this clinical presentation to the attention of clinicians. We emphasize the morphology, histopathology, immunohistochemical profiles, and genetic mutations associated with these cases.
Materials And Methods: A systematic review of the National Center for Biotechnology Information's PubMed was conducted, utilizing the following specific key words to identify all adult LCH patients with cutaneous intertriginous involvement: "Intertriginous Langerhans," "Vulvar Langerhans," "Genital Langerhans," "Perineal Langerhans," "Perianal Langerhans," "Intergluteal Langerhans," "Inguinal Langerhans," "Axillary Langerhans," and "Inframammary Langerhans.
N Engl J Med
January 2025
From the Department of Medicine, Northwestern Memorial Hospital, Chicago (C.W.Y.); and the Departments of Medicine (J.S.G., A.J.Y.), Radiology (B.G.G.), and Pathology (B.M.H.), Massachusetts General Hospital, the Departments of Medicine (J.S.G., R.H.F., A.J.Y.), Radiology (B.G.G.), and Pathology (B.M.H.), Harvard Medical School, and the Department of Medicine, Brigham and Women's Hospital (R.H.F.) - all in Boston.
N Engl J Med
January 2025
Assistance Publique-Hôpitaux de Paris-Sorbonne Université, Paris, France
Genet Test Mol Biomarkers
January 2025
Department of Biology, University of Sistan and Baluchestan, Zahedan, Iran.
Fanconi anemia (FA) is a rare genetic disorder that affects multiple systems in the body and is the most prevalent congenital syndrome, leading to bone marrow failure. Twenty-two genes have been identified as contributors to the disease. Significant advancements have been made in the past 2 decades in understanding the genetic and pathophysiological processes involved.
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