Introduction: Behçet's disease (BD) is a chronic systemic condition characterized by recurrent oral and genital ulcers, neurological and vascular manifestations, ocular and skin lesions, and a positive pathergy test. Mouth and genital ulcers with inflamed cartilage (MAGIC) syndrome is rare and consists of features of BD together with relapsing polychondritis. The treatment of this syndrome is similar that of BD and depends on disease expression and symptoms.
Case Report: We report the case of a 50-year-old man diagnosed with BD with relapsing polychondritis (MAGIC syndrome) which had evolved over approximately 35 years, with exuberant and frequent oral and genital ulcers. Various treatments were unsuccessful and had adverse effects. Lenalidomide was initiated at a dose of 5 mg per day with remission of oral and genital ulcers and no haematological or adverse effects.
Conclusion: BD is a complex disorder with a variety of presentations. Its treatment can be challenging but sometimes off-label drugs, like lenalidomide, can be effective.
Learning Points: Behçet's disease (BD) is a chronic systemic autoinflammatory disease whose treatment can be challenging and is dependent on disease expression and symptoms.Thalidomide can be effective for treating recurrent oral aphthae, but its use is limited by adverse effects, mostly sensory polyneuropathy.Lenalidomide is a more powerful thalidomide analogue with fewer adverse effects and may be effective for the treatment of BD.
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http://dx.doi.org/10.12890/2019_001117 | DOI Listing |
BMC Pregnancy Childbirth
January 2025
Department of Obstetrics and Gynecology, The Affiliated Traditional Chinese Medicine Hospital, Southwest Medical University, Longmatan Disrict, Chunhui Road 182, Luzhou, Sichuan, 646000, China.
Background: Behçet's disease (BD) during pregnancy is a relatively rare condition, and there are currently no established guidelines for its management. The effects of BD on both mothers and children remain unclear. In this paper, we present the diagnostic and treatment processes for a patient with BD during pregnancy.
View Article and Find Full Text PDFFront Immunol
January 2025
Department of Rheumatology & Allergology, Japanese Red Cross Medical Center, Tokyo, Japan.
Patients with A20 haploinsufficiency (HA20) presenting with central nervous system (CNS) symptoms are rare, and available reports are limited. Here, we describe a patient with HA20, previously followed up as Behçet disease, who presented with CNS symptoms in adulthood. A 38-year-old Japanese male who had been followed up for incomplete Behçet disease at another hospital since 28 years of age presented to our hospital with acute-onset diplopia and persistent hiccups that were severe enough to cause vomiting.
View Article and Find Full Text PDFJ 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.
Int J STD AIDS
January 2025
Genitourinary and HIV Medicine and BASHH Clinical Effectiveness Group, Central North West London NHS Foundation Trust, London, UK.
Background: The management of vulval disorders in Genitourinary Medicine (GUM) clinics requires targeted approaches due to the wide range of conditions affecting the vulva. Vulval diseases encompass various aetiologies, including dermatoses, pain syndromes, and pre-malignant conditions, necessitating specialized care often involving multidisciplinary collaboration.
Purpose: This guideline aims to provide evidence-based recommendations for the diagnosis and management of specific vulval conditions that may present in GUM clinics.
J Infect Dev Ctries
December 2024
Faculty of Medicine, Alexandria National University, Egypt.
Introduction: Herpes simplex virus type-2 (HSV-2) infection is a sexually transmitted disease (STD) that causes genital ulcers. The prevalence of HSV-2 increases because of its asymptomatic shedding. This study aimed to evaluate community knowledge and attitude toward HSV-2 infection in Al-Jouf region.
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