eMedicine Specialties > Pediatrics: General Medicine > Rheumatology

Weber-Christian Disease: Differential Diagnoses & Workup

Author: Moise L Levy, MD, Professor, Departments of Pediatrics and Dermatology, Baylor College of Medicine; Chief, Department of Dermatology, Texas Children's Hospital
Contributor Information and Disclosures

Updated: May 8, 2009

Differential Diagnoses

Polyarteritis Nodosa
Sarcoidosis
Systemic Lupus Erythematosus
Systemic Sclerosis
Vasculitis and Thrombophlebitis

Other Problems to Be Considered

Alpha1-antitrypsin deficiency panniculitis
Cutaneous polyarteritis nodosa
Eosinophilic fasciitis
Eosinophilic myalgia syndrome
Erythema induratum
Erythema nodosum
Leukemia
Lipodermatosclerosis
Lobular panniculitis
Lymphoma
Pancreatic panniculitis
Poststeroid panniculitis
Sclerema neonatorum
Scleroderma panniculitis
Septal panniculitis
Superficial migratory thrombophlebitis

Workup

Laboratory Studies

  • Changes in liver function test results, CBC count, and electrolyte levels reflect visceral involvement of organs, including the lungs, heart, intestines, spleen, kidneys, and adrenal glands in patients with Weber-Christian disease.
  • Patients may present with a leukocytosis or leukopenia, anemia, or hypocomplementemia.
  • The erythrocyte sedimentation rate is usually elevated, although the degree of elevation varies.
  • Serum and urine amylase and lipase levels are within the reference range, differentiating Weber-Christian disease from a panniculitis associated with pancreatic disease.
  • The alpha1-antitrypsin level is within the reference range, 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).

    Standard posteroanterior chest radiograph reveals...

    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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    Standard posteroanterior chest radiograph reveals...

    Standard posteroanterior chest radiograph reveals extensive bilateral hilar and mediastinal lymph node enlargement not associated with a pulmonary abnormality in a patient with sarcoidosis.


    Young male patient with fever and cough has a foc...

    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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    Young male patient with fever and cough has a foc...

    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.

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
      • Poststeroid 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.

More on Weber-Christian Disease

Overview: Weber-Christian Disease
Differential Diagnoses & Workup: Weber-Christian Disease
Treatment & Medication: Weber-Christian Disease
Follow-up: Weber-Christian Disease
Multimedia: Weber-Christian Disease
References
Further Reading
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References

  1. Weber EP. A case or relapsing nonsuppurative nodular panniculitis. Brit J Derm. 1925;37:301.

  2. Christian HA. Relapsing febrile nodular nonsuppurative panniculitis. Arch Intern Med. 1928;41:338.

  3. Haubrich WS. Weber and Christian of Weber-Christian disease. Gastroenterology. Apr 2008;134(4):912. [Medline].

  4. Valverde R, Rosales B, Ortiz-de Frutos FJ, Rodriguez-Peralto JL, Ortiz-Romero PL. Alpha-1-antitrypsin deficiency panniculitis. Dermatol Clin. Oct 2008;26(4):447-51, vi. [Medline].

  5. Wu F, Zou CC. Childhood Weber-Christian disease: clinical investigation and virus detection. Acta Paediatr. Nov 2007;96(11):1665-9. [Medline].

  6. Sharma AK, Sharma PR. Idiopathic lobular panniculitis (Weber Christian disease): a case report. Kathmandu Univ Med J (KUMJ). Apr-Jun 2006;4(2):243-5. [Medline].

  7. Abuzahra F, Kovacs S, Beermann T, et al. Treatment of relapsing idiopathic nodular panniculitis with clofazimine. Br J Dermatol. Mar 2005;152(3):582-3. [Medline].

  8. Eravelly J, Waters MF. Thalidomide in Weber-Christian disease. Lancet. Jan 29 1977;1(8005):251. [Medline].

  9. Freedberg IM, Eisen AZ, Wolff K. Panniculitis. In: Fitzpatrick's Dermatology in General Medicine. Vol 1. New York, NY: Mc-Graw Hill; 1999:1275-8.

  10. Hood AF, Kwan TH, Mihm ML, Jr. Panniculitis. In: Primer of Dermatopathology. Boston, MA: Little, Brown and Company; 1993:450.

  11. Lazarus GS. Panniculitis and Disorders of the Subcutaneous Fat. [Full Text].

  12. Lebwohl M. Panniculitis. In: Difficult Diagnoses in Dermatology. New York, NY: Churchill Livingstone; 1988:389-91.

  13. Lemley DE, Ferrans VJ, Fox LM, et al. Cardiac manifestations of Weber-Christian disease: report and review of the literature. J Rheumatol. May 1991;18(5):756-60. [Medline].

  14. Moschella SL, Hurley, HJ. Panniculitides. In: Dermatology. Vol 2. Philadelphia, PA: WB Saunders; 1985:1175-6.

  15. Panush RS, Yonker RA, Dlesk A, et al. Weber-Christian disease. Analysis of 15 cases and review of the literature. Medicine (Baltimore). May 1985;64(3):181-91. [Medline].

  16. Schuval SJ, Frances A, Valderrama E, et al. Panniculitis and fever in children. J Pediatr. Mar 1993;122(3):372-8. [Medline].

  17. Usuki K, Kitamura K, Urabe A, Takaku F. Successful treatment of Weber-Christian disease by cyclosporin A. Am J Med. Aug 1988;85(2):276-8. [Medline].

  18. White JW Jr, Winkelmann RK. Weber-Christian panniculitis: a review of 30 cases with this diagnosis. J Am Acad Dermatol. Jul 1998;39(1):56-62. [Medline].

  19. Winkelmann RK, Dahl PR, Perniciaro C, Dahl PM. Asteroid bodies and other cytoplasmic inclusions in necrobiotic xanthogranuloma with paraproteinemia. J Am Acad Dermatol. Jun 1998;38(6 Pt 1):967-70. [Medline].

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Keywords

Weber-Christian disease, idiopathic lobular panniculitis, relapsing febrile nodular nonsuppurative panniculitis, nodular nonsuppurative panniculitis, Pfeifer-Weber-Christian syndrome, lupus panniculitis, factitial panniculitis, pancreatic disease, histiocytic cytophagic panniculitis, skin inflammation, skin rash, skin lesions, hepatomegaly, splenomegaly, treatment, diagnosis

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Contributor Information and Disclosures

Author

Moise L Levy, MD, Professor, Departments of Pediatrics and Dermatology, Baylor College of Medicine; Chief, Department of Dermatology, Texas Children's Hospital
Moise L Levy, MD is a member of the following medical societies: American Academy of Dermatology, American Academy of Pediatrics, American Society for Laser Medicine and Surgery, Harris County Medical Society, Society for Investigative Dermatology, and Texas Medical Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Thomas JA Lehman, MD, FAAP, FACR, Clinical Professor of Pediatrics, Department of Pediatrics, Division of Pediatric Rheumatology, Weill-Cornell University; Chief, Hospital for Special Surgery
Thomas JA Lehman, MD, FAAP, FACR is a member of the following medical societies: PM American Allergy Society
Disclosure: Nothing to disclose.

CME Editor

Daniel Rauch, MD, FAAP, Director, Pediatric Hospitalist Program, Associate Professor, Department of Pediatrics, New York University School of Medicine
Daniel Rauch, MD, FAAP is a member of the following medical societies: Ambulatory Pediatric Association, American Academy of Pediatrics, and Society of Hospital Medicine
Disclosure: Baxter Honoraria Consulting

Chief Editor

Lawrence K Jung, MD, Chief, Division of Pediatric Rheumatology, Children's National Medical Center
Lawrence K Jung, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Rheumatology, Clinical Immunology Society, and New York Academy of Sciences
Disclosure: Nothing to disclose.

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