Weber Christian disease is a term used to describe idiopathic nodular or lobular panniculitis, and is characterized by lobular panniculitis (inflammation within the fat lobules), subcutaneous nodules and systemic symptoms. Before making a diagnosis of idiopathic lobular panniculitis, other more specific diagnoses (e.g. lupus panniculitis, alpha1-antitrypsin deficiency panniculitis, pancreatic panniculitis) should be ruled out.

Histologically, there is inflammation of the lobules, with the presence of neutrophils in early lesions and abundant foamy histiocytes in later lesions, accompanied by fat necrosis (Rapini). Note that this entity may be difficult to distinguish from alpha1-antitrypsin deficiency panniculitis and in fact, cases of alpha1-antitrypsin deficiency panniculitis may have been diagnosed as Weber Christian disease in the past. However, a simple lab test testing for levels of alpha1-antitrypsin will help resolve the issue.

Erythematous, tender nodules are usually on the legs, accompanied by systemic symptoms (fever, malaise, arthralgias). Laboratories tests may reveal abnormalities in liver function tests, CBC, and electrolyte levels as WCD can involve the lungs, heart, intestines, spleen, kidneys and adrenal glands.

→WCD is an idiopathic lobular panniculitis with systemic symptoms.

→Histologically characterized by inflammation in the fat lobules composed of neutrophils (early lesions) and foamy macrophages (later lesions).

→Must be distinguished from specific causes of lobular panniculitis such as alpha1-antitrypsin deficiency panniculitis.