Weber-Christian disease
Overview
Weber-Christian disease is a skin condition that features recurring inflammation in the fat layer of the skin. The involved areas of skin manifest as recurrent crops of erythematous, sometimes tender, edematous subcutaneous nodules. The lesions are symmetric in distribution, and the thighs and lower legs are affected most frequently. Malaise, fever, and arthralgias frequently occur. Nausea, vomiting, abdominal pain, weight loss, and hepatomegaly may also occur. Because its etiology is unknown, Weber-Christian disease is often referred to as idiopathic lobular panniculitis.
Symptoms
Patients affected with Weber-Christian disease describe crops of lesions that appear and resolve during a period of weeks to months. The lesions are often symmetric in distribution, and the thighs and legs are involved most commonly. Individual nodules regress during the course of a few weeks. Systemic symptoms of Weber-Christian disease include malaise, fever, nausea, vomiting, abdominal pain, weight loss, bone pain, myalgia, and arthralgia.
Causes
Because its etiology is unknown, Weber-Christian disease is called idiopathic lobular panniculitis. Patients with Weber-Christian disease do not report a history of physical trauma. In some patients with Weber-Christian disease, elevated levels of circulating immune complexes have been noted, suggesting an immunologically mediated reaction. Similarities between Weber-Christian disease and alpha1-antitrypsin deficiency suggest that an altered regulation of a normal inflammatory process may be involved.
Prognosis
Prognosis for Weber-Christian disease: spontaneous regression usually after a few weeks then frequent relapses occur within months or years