The reported case belongs in rarely occurring general diseases running a chronic relapsing course. The case is uncommon because of not only general affection of the adipose tissue presenting with immunologic inflammation, but also of the development of manifest abnormal changes in the internals, amyloidosis in particular. 20-year follow-up of the patient suggests that the evolution of the above pathological processes might be caused by both the nature of the underlying disease and a long-continued administration of corticosteroids.
Download full-text PDF |
Source |
---|
Dermatol Clin
April 2024
Mayo Clinic Department of Dermatology, 200 First Street Southwest, Rochester, MN 55905, USA.
Neutrophilic panniculitides are a heterogeneous group of inflammatory disorders encompassing many different entities. This review article focuses on the epidemiology, pathogenesis, clinicopathological features, diagnosis, and treatment of selected diseases. Patients often seek care due to systemic involvement, but the variable presentation of panniculitides can present a diagnostic challenge.
View Article and Find Full Text PDFAm J Case Rep
January 2021
Department of Internal Medicine, Jeju National University Hospital, Jeju City, Jeju, South Korea.
BACKGROUND Pfeifer-Weber-Christian disease (PWCD), also referred to as idiopathic nodular panniculitis, is a rare idiopathic disease characterized by lobular panniculitis of adipose tissue with systemic symptoms and multiple organ involvement and is usually treated with corticosteroids and cyclosporine A. We report a case of PWCD that was unresponsive to standard treatment but responded to intravenous immune globulin (IVIG) therapy. CASE REPORT A 35-year-old Korean woman presented with fever, malaise, myalgia, and painful nodules in the left breast.
View Article and Find Full Text PDFClin Med Insights Case Rep
May 2020
Rheumatology Unit, Department of Medical and Surgical Sciences, University of Foggia, Foggia, Italy.
Pfeifer-Weber-Christian disease (PWCD) is a rare idiopathic disease characterized by lobular panniculitis of adipose tissue with systemic symptoms and multiple organ involvement. Even though the systemic involvement is rare, it is life-threatening and represent a treatment challenge for the clinicians. We report a case of PWCD characterized by hepatic, hematologic, and renal involvement, with good response to mofetil mycophenolate and prednisone treatment.
View Article and Find Full Text PDFBMJ Case Rep
May 2019
Department of General Medicine, Singhealth, Singapore.
Inflammatory conditions manifest with a broad spectrum of signs and symptoms. Panniculitis is such a condition affecting the subcutaneous fat and presents as tender erythematous nodules. It is also associated with a systemic response and has been described in the literature as early as in 1892 by Pfeifer and in the 1920s by Weber and Christian.
View Article and Find Full Text PDFIndian J Dermatol
October 2016
Department of Dermatology and Venereology, Andreas Sygros Skin Hospital, Athens, Greece. E-mail:
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!