J W White1, R K Winkelmann. 1. Department of Dermatology, Mayo Clinic Jacksonville, Florida 32224, USA.
Abstract
BACKGROUND: More than 60 years ago, Christian described a panniculitis that was later termed Weber-Christian disease. OBJECTIVE: The purpose of this study was to investigate whether this is a specific disease or a nonspecific disease that embraces several specific conditions. METHODS: We studied 30 cases diagnosed as Weber-Christian panniculitis and found it possible to make a more specific diagnosis. RESULTS: In 12 patients, findings were compatible with erythema nodosum. Six patients had phlebitis or postphlebitic syndrome. Factitial panniculitis was diagnosed in five patients, and trauma had a role in the conditions of another three patients. Cytophagic panniculitis, lymphoma, and leukemia were recognized in one patient each. The lesion was lobular in almost all cases, and the presence of lipophagia was noted in 19 biopsy specimens. Granulomatous, neutrophilic, and lymphocytic pathologic changes were present in nine, eight, and eight tissue specimens, respectively. CONCLUSION: The recognition of distinct disease patterns of fat lesions as fat necrosis with pancreatic disease, alpha1-antitrypsin panniculitis, lupus and connective tissue disease panniculitis, involution lipoatrophy, lipomembranous panniculitis, factitial panniculitis syndromes, calcification panniculitis, lipophagic lipoatrophy, and cytophagic panniculitis has lessened the need for a less specific panniculitis category. All these diseases have been reported in the literature as "Weber-Christian disease." Because separate and distinct forms of fat lesions have been described, we believe that the eponym should be abandoned and that more specific diagnoses should be made on the basis of pathogenesis or cause.
BACKGROUND: More than 60 years ago, Christian described a panniculitis that was later termed Weber-Christian disease. OBJECTIVE: The purpose of this study was to investigate whether this is a specific disease or a nonspecific disease that embraces several specific conditions. METHODS: We studied 30 cases diagnosed as Weber-Christian panniculitis and found it possible to make a more specific diagnosis. RESULTS: In 12 patients, findings were compatible with erythema nodosum. Six patients had phlebitis or postphlebitic syndrome. Factitial panniculitis was diagnosed in five patients, and trauma had a role in the conditions of another three patients. Cytophagic panniculitis, lymphoma, and leukemia were recognized in one patient each. The lesion was lobular in almost all cases, and the presence of lipophagia was noted in 19 biopsy specimens. Granulomatous, neutrophilic, and lymphocytic pathologic changes were present in nine, eight, and eight tissue specimens, respectively. CONCLUSION: The recognition of distinct disease patterns of fat lesions as fat necrosis with pancreatic disease, alpha1-antitrypsinpanniculitis, lupus and connective tissue disease panniculitis, involution lipoatrophy, lipomembranous panniculitis, factitial panniculitis syndromes, calcification panniculitis, lipophagic lipoatrophy, and cytophagic panniculitis has lessened the need for a less specific panniculitis category. All these diseases have been reported in the literature as "Weber-Christian disease." Because separate and distinct forms of fat lesions have been described, we believe that the eponym should be abandoned and that more specific diagnoses should be made on the basis of pathogenesis or cause.
Authors: Georg Pongratz; Boris Ehrenstein; Wolfgang Hartung; Jürgen Schölmerich; Martin Fleck Journal: BMC Musculoskelet Disord Date: 2010-01-27 Impact factor: 2.362