A patient with Weber-Christian panniculitis is described in this report in which treatment with prednisone and hydroxychloroquine caused no improvement of the disease, and even led to a worsening of the symptoms. In contrast, administration of oral cyclophosphamide led to a rapid remission of the disease. As Weber-Christian disease has no known aetiology and no specific treatment has been established, the successful therapy with the cytostatic drug cyclophosphamide may shed light on the pathogenesis of Weber-Christian panniculitis.
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http://dx.doi.org/10.1007/BF00541342 | 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 PDFBeijing Da Xue Xue Bao Yi Xue Ban
December 2022
Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing 100044, China.
Hemophagocytic syndrome (HPS) is a severe disease characterized by excessive release of inflammatory cytokines caused by abnormal activation of lymphocytes and macrophages, which can cause multiple organ damage and even death. Panniculitis is a disease characterized by inflammation of subcutaneous adipose tissue. We effectively treated 2 patients with panniculitis-associated HPS with ruxolitinib.
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.
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