History
Patients with Weber-Christian disease present with cutaneous and systemic complaints. It is described as a relatively severe relapsing chronic condition.
Patients 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 most commonly involved. Individual nodules regress over the course of a few weeks.
Systemic symptoms of Weber-Christian disease include fever, malaise, nausea, vomiting, abdominal pain, weight loss, bone pain, myalgia, and arthralgia.
The etiology of Weber-Christian disease is unknown. Patients do not report a history of thermal, mechanical, or chemical trauma.
Physical Examination
Physical examination reveals erythematous, edematous, and tender subcutaneous nodules. See the images below.
Lesion of erythema nodosum: tender, erythematous, nodular lesions located over the extensor surfaces of the legs.
Lesion of Weber-Christian disease: tender, erythematous, nodular lesions located over the limbs with cutaneous atrophy.
The nodules are usually symmetric and measure approximately 1-2 cm; however, nodules may be much larger. The lesions commonly occur on the thighs and lower legs, and may also involve the arms, trunk, and face.
Individual nodules resolve over a 2-week period, leaving an atrophic depressed scar.
Occasionally, the epidermis overlying the nodules breaks down, and the lesion discharges a brown liquid oil (ie, liquefying panniculitis).
Hepatomegaly or splenomegaly may be present in Weber-Christian disease in individuals with visceral involvement.
Although orbital Weber-Christian disease is rare, ophthalmologists need to be aware that patients with Weber-Christian disease can develop severe ocular inflammation. [17, 18]
Complications
Weber-Christian disease may involve the lungs, heart, intestines, spleen, kidney, and adrenal glands. Death may occur in patients with inflammation involving these critical visceral organs.
In patients with primarily cutaneous manifestations, the clinical course may be characterized by exacerbations and remissions of the cutaneous lesions for several years before the disorder subsides.
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Lesion of erythema nodosum: tender, erythematous, nodular lesions located over the extensor surfaces of the legs.
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Standard posteroanterior chest radiograph reveals extensive bilateral hilar and mediastinal lymph node enlargement not associated with a pulmonary abnormality in a patient with sarcoidosis.
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Young male patient with fever and cough has a focal opacity in the left lower lobe that looks like a pneumonia. This is a case of primary tuberculosis.
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Lesion of Weber-Christian disease: tender, erythematous, nodular lesions located over the limbs with cutaneous atrophy.
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A portion of skin is examined in multiple sections and at various magnifications. The epidermis is intact; however, it is infiltrated by small numbers of lymphocytes. A mild infiltrate of lymphocytes and histiocytes are present in the upper dermis. The most prominent change is in the subcutaneous tissue, where a prominent infiltrate of histiocytes, smaller numbers of lymphocytes, and a few plasma cells in the subcutaneous adipose tissue are noted. Occasional foam cells are also evident, and, in places, histocytes surround lipid cysts. Small clusters of necrotic cells and scattered nuclear dust are noted. Minimal extension of this infiltrate into adjacent dense collagenous tissue is observed. (Courtesy of Milton J. Finegold, MD, Professor of Pathology and Pediatrics, Baylor College of Medicine, Houston, TX).
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Magnification of previous specimen X 100.
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Magnification of previous specimen X 200.
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Histopathologic features of alpha-1-antitrypsin deficiency panniculitis. (A) Scanning power shows a mostly lobular panniculitis. (B) Aggregations of neutrophils within the fat lobule are seen. (C) Neutrophils are interstitially arranged between collagen bundles of the deep reticular dermis. (A-C, hematoxylin-eosin stain; original magnifications: A, X 20; B, X 400; C, X 200).
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Histopathologic features of late stage lesions of traumatic panniculitis. This lesion corresponds to the so-called nodular cystic fat necrosis or mobile encapsulated lipoma. A, Scanning power shows encapsulated and well-circumscribed lesion with no inflammatory infiltrate (arrow indicates area enlarged in B). B, At periphery of the lesion necrotic adipocytes appear as anucleated fat cells. (A and B, Hematoxylin-eosin stain; original magnifications: A, ×20; B, ×200.)
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Histopathologic features of paraffinoma. A, Scanning power shows a mostly lobular panniculitis (arrow indicates area enlarged in B). B, Higher magnification demonstrates cystic spaces within the fat lobule surrounded by foamy histiocytes. (A and B, hematoxylin-eosin stain; original magnifications: A, ×20; B, ×200.)
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Histopathologic features of subcutaneous fat necrosis of the newborn. (A) Scanning power shows a mostly lobular panniculitis (arrow indicates area enlarged in B). (B) Higher magnification demonstrated narrow needle-shaped clefts radially arranged and surrounded by histiocytes. (A and B, hematoxylin-eosin stain; original magnifications: A, X 20; B, X 200).
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Histopathologic features of lipoatrophy secondary to subcutaneous injections of corticosteroids. (A) Low-power view showed small fat lobules (arrow indicates area enlarged in B). (B) Higher magnification demonstrates small adipocytes and prominent capillary proliferation, resembling embryonic fat. (A and B, hematoxylin-eosin stain; original magnifications: A, X 20; B, X 200).
