eMedicine Specialties > Pediatrics: General Medicine > Rheumatology

Weber-Christian Disease

Author: Moise L Levy, MD, Professor, Departments of Pediatrics and Dermatology, Baylor College of Medicine; Chief, Department of Dermatology, Texas Children's Hospital
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Updated: May 8, 2009

Introduction

Background

In 1892, Pfeifer first described the skin condition now known as Weber-Christian disease, or idiopathic lobular panniculitis. In 1925, Weber further depicted the syndrome.¹ In 1928, Christian emphasized the significance of fever as part of the syndrome. Henceforth, the syndrome became known as Weber-Christian disease.^2,3 The nomenclature of this and other related diseases is confusing, and some authors believe that the eponym should be abandoned and that more specific diagnoses should be made on the basis of pathogenesis or cause.

Diseases such as lupus panniculitis, factitial panniculitis, panniculitis associated with pancreatic disease, histiocytic cytophagic panniculitis, and alpha1-antitrypsin deficiency panniculitis have been differentiated from Weber-Christian disease.⁴ As Weber-Christian disease is elucidated further, additional diseases will probably be identified as being distinct from Weber-Christian disease.

Pathophysiology

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.^5,6

Frequency

United States

Because of the ambiguity of this diagnosis versus other closely related conditions, the frequency of Weber-Christian disease has not been determined.

Mortality/Morbidity

The course of Weber-Christian disease varies and depends on which organs are affected.

• Weber-Christian disease may involve the lungs, heart, intestines, spleen, kidney, and adrenal glands. In patients with inflammation involving visceral organs, significant morbidity and mortality may occur.
• In patients with only cutaneous manifestations, the clinical course may be characterized by exacerbations and remissions of the cutaneous lesions for several years before the disorder subsides.

Race

No racial predilection is apparent.

Sex

The disease occurs more often in women, who comprise approximately 75% of reported cases.

Age

Weber-Christian disease may occur in young children but has been reported most frequently in people in the fourth to seventh decades of life.

Clinical

History

Patients with Weber-Christian disease typically have cutaneous and, less frequently, systemic 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.
• The etiology of Weber-Christian disease is unknown, and patients do not report a history of thermal, mechanical, or chemical trauma.

Physical

Physical examination reveals erythematous, edematous, and tender subcutaneous nodules.

• The nodules are usually symmetric and measure approximately 1-2 cm; however, the nodules may be much larger. The lesions commonly occur on the thighs and lower legs but may also involve the arms, trunk, and face.
• The individual nodules resolve during a couple of weeks, leaving an atrophic depressed scar.
• Occasionally, the epidermis overlying the nodules breaks down, and the lesion discharges a brown liquid oil (ie, liquefying panniculitis).
• In individuals with Weber-Christian disease with visceral involvement, hepatomegaly or splenomegaly may be present.

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.

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