Weber-Christian disease: a case report.

نویسندگان

  • C POKORNY
  • C A HELLWIG
چکیده

Weber-Christian disease is a rare and poorly understood disorder of the adipose tissue.1,2 The exact aetiopathogenesis of this condition is largely unknown and it is also called idiopathic lobular panniculitis.3 In 1892, Pfeifer first described the skin condition now known as Weber-Christian disease, or idiopathic lobular panniculitis.4,5 In 1925, Weber further depicted the syndrome as case of "relapsing non-suppurative nodular panniculitis".6,7 In 1928, Christian emphasized the significance of fever as a part of the syndrome.8 Henceforth, the syndrome is known as Weber-Christian disease. WCD may occur in young children, but it mostly affects people from fourth to seventh decades of life. The skin manifestation is reflected by recurrent crops of erythematous, sometimes tender and edematous subcutaneous nodules. The lesions are symmetric in distribution and predominantly involve the lower extremities but lesions can also occur on the trunk, upper extremities and rarely on the face.9 The lesions resolve in a couple of weeks, leaving an atrophic depressed scar.10 Malaise, fever, and arthralgia frequently occur. Nausea, vomiting, abdominal pain, weight loss, bone pain, myalgia, hepatomegaly and or splenomegaly may also occur. WCD may involve the internal organs like lungs, heart, intestines, spleen, kidney, and adrenal glands. In patients with inflammation involving visceral organs, significant morbidity and mortality may occur.11 WCD being a rare condition and our experience of good therapeutic response to corticosteroid prompted us to report this case and highlight the clinical and diagnostic features.

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عنوان ژورنال:
  • The Journal of the Kansas Medical Society

دوره 55 2  شماره 

صفحات  -

تاریخ انتشار 1954