ABSTRACT
Objective To analyze the clinical features of Weber-Christian disease (WCD) and to make a review of the literature for early diagnosis and treatment.Methods The clinical features of an atypical WCD patient who had been misdiagnosed as polymyositis were analyzed.Results WCD was characterized by subcutaneous nodules and systemic symptoms.Repeating physical examination and biopsy in time were important if the nodules were not obvious.Conclusion WCD is often misdiagnosed because of the complicated clinical manifestations.Carefully physical examination and timely biopsy are help for early diagnosis.
ABSTRACT
Weber-Christian disease (WCD) is a rare inflammatory disease of adipose tissue, which is characterized by painful cutaneous nodules and constitutional symptoms. Although any area of the body containing fat can be affected by WCD, the involvement of retrobulbar fat is uncommon and proptosis is a rare presenting manifestation. We report a case who presented with proptosis of the right eye which is accompanied by painful subcutaneous nodules, high fever and myalgia. Biopsies of retrobulbar tissue and suprapubic nodule showed lobular panniculitis with mixed cellular infiltration, mainly composed of histiocytes and lymphocytes. He responded well to high-dose glucocorticoid.
Subject(s)
Adult , Humans , Male , Biopsy , Exophthalmos/pathology , Exophthalmos/etiology , Exophthalmos/drug therapy , Glucocorticoids/administration & dosage , Magnetic Resonance Imaging , Panniculitis, Nodular Nonsuppurative/pathology , Panniculitis, Nodular Nonsuppurative/drug therapy , Panniculitis, Nodular Nonsuppurative/complicationsABSTRACT
Weber-Christian disease (WCD) is a rare inflammatory disease of adipose tissue, which is characterized by painful cutaneous nodules and constitutional symptoms. Although any area of the body containing fat can be affected by WCD, the involvement of retrobulbar fat is uncommon and proptosis is a rare presenting manifestation. We report a case who presented with proptosis of the right eye which is accompanied by painful subcutaneous nodules, high fever and myalgia. Biopsies of retrobulbar tissue and suprapubic nodule showed lobular panniculitis with mixed cellular infiltration, mainly composed of histiocytes and lymphocytes. He responded well to high-dose glucocorticoid.