Weber-Christian panniculitis in children : it is related to some specific diseases

Objective More than half a century ago, Weber and Christian described a panniculitis that was later termed Weber-Christian disease. Recently, it was recognized that a variety of distinctive disease entities are associated with Weber-Christian disease.The most researchers consider that the term "Weber-Christian disease" should be abandoned and more specific diagnoses should be made on the basis of pathogenesis or cause. The purpose of this study was to investigate whether Weber-Christian panniculitis in children is a specific or nonspecific disease. Methods The authors studied 16 cases diagnosed as Weber-Christian panniculitis at the rheumatologic clinic, Beijing Children′s Hospital from Jan 1993 to Feb 2000. The ratio of boy to girl was 7∶9. The mean age of onset was 5.1 years. The average course of the disease was 10.8 months. Based on clinical manifestations, routine pathological and immunohistochemical methods, results of laboratory examination and follow-up data, the arthors found that it was possible to make a more specific diagnosis. Result A variety of distinctive disease entities were recognized in the 16 children, including 7 subcutaneous panniculitis-like T-cell lymphoma (SPTCL); systemic lupus erythematosis (SLE), systemic cryptococcosis, inflammatory bowel disease (IBD) and malignant histiocytosis in one patient each; tuberculosis and streptococcus infection were diagnosed in two children each. The cause was unclear in one case. Thirteen of 16 children were followed-up. Four cases, tuberculosis or streptococcus infection, were cured with antibiotics and antituberculosis agents. SLE and IBD were stable with regular treatment. One SPTCL case was unstable who were treated with irregular prednisone.Six cases died, including 3 with SPTCL, one with malignant histiocytosis and one with systemic cryptococcosis, one with unknown cause. Conclusion Weber-Christian panniculitis seems not to be a specific disease.It is rather a nonspecific subcutaneous inflammation. A variety of distinctive diseases are associated with Weber-Christian disease. It is proposed that the term "Weber-Christian panniculitis" should be abandoned and that more specific diagnosis should be made on the base of pathogenesis or causes.