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 also may occur. Because its etiology is unknown, Weber-Christian disease often is referred to as idiopathic lobular panniculitis.

Frequency:
 

• In the US: 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 is variable 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 appears to exist.

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

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.

• 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 also may involve the arms, trunk, and face.

• The individual nodules resolve during a couple of weeks and leaving an atrophic depressed scar.

• Occasionally, the epidermis overlying the nodules breaks down, and the lesion discharges a brown liquid oil (i.e. 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.

Lab Studies:

• Changes in liver function tests, complete blood count, and electrolytes reflect visceral involvement of organs, including the lungs, heart, intestines, spleen, kidneys, and adrenal glands.

• Patients may present with a leukocytosis or leukopenia, anemia, or hypocomplementemia.

• The erythrocyte sedimentation rate usually is elevated, although the degree of elevation varies.

• Serum and urine amylase and lipase levels are normal, differentiating Weber-Christian disease from a panniculitis associated with pancreatic disease.

• The alpha1-antitrypsin level is normal, differentiating Weber-Christian disease from alpha1-antitrypsin panniculitis.

Imaging Studies:

• Obtain a chest radiograph to exclude autoimmune diseases (eg, sarcoidosis) and infectious diseases (eg, tuberculosis).

Procedures:

• Skin biopsy is necessary to confirm the diagnosis of panniculitis.

Histologic Findings:

Classification of panniculitis based on histologic criteria

Lobular panniculitis

• Without vasculitis
  • Idiopathic lobular panniculitis (Weber-Christian disease)
  • Histiocytic cytophagic panniculitis
  • Alpha1-antitrpysin deficiency panniculitis
  • Physical panniculitis
    • Cold induced
    • Traumatic
    • Chemical induced
    • Factitial
  • Neonatal panniculitis
    • Sclerema neonatorum
    • Neonatal subcutaneous fat necrosis
    • Post-steroid panniculitis
  • Lobular panniculitis of systemic disease
    • Pancreatic panniculitis
    • Lupus erythematosus
    • Sarcoidosis
    • Calcifying panniculitis of renal failure
    • Lymphoma and leukemia
    • Infections
• With vasculitis
  • Nodular vasculitis (erythema induratum)

Septal panniculitis

• Without vasculitis
  • Erythema nodosum
  • Scleroderma panniculitis
  • Lipodermatosclerosis
  • Eosinophilic fasciitis
  • Eosinophilic myalgia syndrome
• With vasculitis
  • Superficial migratory thrombophlebitis
  • Polyarteritis nodosa
  • Cutaneous polyarteritis nodosa

Three histopathologic stages observed in Weber-Christian disease

• The first stage is characterized by an acute inflammatory reaction, in which lobules of fat are replaced by neutrophils, lymphocytes, and histiocytes.
• In the second stage, macrophages migrate and phagocytose degenerated fat, forming characteristic "foam cells."
• In the third stage, the foam cells are replaced by fibroblasts, and the inflammatory reaction is replaced by fibrotic tissue.

Medical Care: No uniformly effective therapy for Weber-Christian disease exists.

• Therapeutic responses have been reported with the use of fibrinolytic agents, hydroxychloroquine, azathioprine, thalidomide, cyclophosphamide, tetracycline, cyclosporine, and mycophenolate.

• Systemic steroids (eg, prednisone) may be effective in suppressing acute exacerbations.

• Nonsteroidal anti-inflammatory agents may reduce fever, arthralgias, and other signs of malaise.

• Involvement of specific organs may require specific supportive drugs.

Surgical Care: No surgical treatment is indicated.

Consultations: Consult dermatology to perform a skin biopsy and to consider the wide variety of causes of panniculitis.

Diet: No specific dietary requirements exist.

No specific uniformly effective therapy for Weber-Christian disease exists. Therapeutic responses have been reported using fibrinolytic agents, hydroxychloroquine, azathioprine, thalidomide, cyclophosphamide, tetracycline, and mycophenolate. Systemic steroids (eg, prednisone) may be effective in suppressing acute exacerbations. Nonsteroidal anti-inflammatory agents (eg, aspirin, indomethacin) may reduce fever, arthralgias, and other signs of malaise. Involvement of specific organs may require specific supportive drugs.
Drug Category: Corticosteroids -- Used for suppression of acute inflammatory exacerbations. These agents have anti-inflammatory properties and cause profound and varied metabolic effects. They modify the body’s immune response to diverse stimuli.

Drug Category: Immunomodulators -- Inhibit key factors that mediate immune reactions, which in turn decrease inflammatory responses. May have potential long-term therapeutic response.

Drug Category: Antibiotics -- Tetracycline has anti-inflammatory activity.

Drug Category: Nonsteroidal anti-inflammatory drugs -- May reduce fever, arthralgia, and pain.

Further Inpatient Care:

• Inpatient hospitalization and supportive care may be necessary in severe incidents of Weber-Christian disease in which inflammation involves visceral organs or for wound care, as indicated.

Further Outpatient Care:

• Monitor individuals with Weber-Christian disease for progression of the disease and for adverse effects of medications. Routine follow-up care is indicated.

In/Out Patient Meds:

• No specific uniformly effective therapy exists for Weber-Christian disease.

• Therapeutic responses have been reported using fibrinolytic agents, hydroxychloroquine, azathioprine, thalidomide, cyclophosphamide, tetracycline, and mycophenolate.

• Systemic steroids (eg, prednisone) may be effective in suppressing acute exacerbations.

• Nonsteroidal anti-inflammatory agents may reduce fever, arthralgias, and other signs of malaise.

• Involvement of specific organs may require specific supportive drugs.

• When the condition subsides, prophylaxis may be unnecessary.

Deterrence/Prevention:

• No effective methods of prevention have been discovered.

Complications:

• Weber-Christian disease may involve the lungs, heart, intestines, spleen, kidney, and adrenal glands. In patients with inflammation involving these critical visceral organs, death 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.

Prognosis:

• The prognosis for patients with Weber-Christian disease is highly variable.

• 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 resolves.

• Patients with severe visceral inflammation of the heart, lungs, intestines, spleen, kidney, or adrenal glands may not survive.

Patient Education:

• Inform patients of the risks and adverse effects of various treatment options.

• Select different treatment modalities on an individual basis.