The authors describe a case of idiopathic nodular panniculitis (Weber-Christian disease) with recurrent febrile episodes resistant to glucocorticosteroids and methotrexate (MTX) in various combinations with hydroxychloroquine, azathioprine, cyclosporine, colchicine, and doxycycline. Thalidomide at 100 mg/day has induced a remission for 3 years.
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http://dx.doi.org/10.1097/00124743-200210000-00005 | DOI Listing |
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 PDFAutoimmune/inflammatory syndrome induced by adjuvants (ASIA) is associated with a hyperergic response of the immune system. The spectrum of clinical changes in ASIA is extensive and difficult to diagnose. Panniculitis is a heterogeneous group of diseases characterized by lesions of the adipose tissue.
View Article and Find Full Text PDFInt J Dermatol
June 2021
Department of Dermatology, American University of Beirut Medical Center, Beirut, Lebanon.
Background: Panniculitides are a heterogeneous group of inflammatory dermatoses involving the subcutaneous fatty tissue. Histologically, they are classified into septal and lobular panniculitis, according to the predominant localization of the inflammatory infiltrate. Neutrophils are frequently found in panniculitis, mainly at the early stages.
View Article and Find Full Text PDFJ Am Acad Dermatol
October 2022
Department of Medicine, Beaumont Hospital, Dublin, Ireland; Irish Centre for Genetic Lung Disease, Royal College of Surgeons in Ireland, Dublin, Ireland.
Background: Panniculitis represents a rare and potentially lethal manifestation of alpha-1 antitrypsin deficiency (AATD). Evidence regarding management is limited to case reports and small case series. We sought to clarify typical features and investigation of AATD-associated panniculitis and assess the evidence regarding therapeutic options.
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.
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