eMedicine Specialties > Pediatrics: General Medicine > Rheumatology

Weber-Christian Disease: Follow-up

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

Follow-up

Further Inpatient Care

  • Inpatient hospitalization and supportive care may be necessary in severe cases 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.

Inpatient & Outpatient Medications

  • No specific uniformly effective therapy for Weber-Christian disease is recognized.
  • Therapeutic responses have been reported using fibrinolytic agents, hydroxychloroquine, azathioprine, thalidomide, cyclophosphamide, tetracycline, mycophenolate, and clofazimine
  • 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 widely varies.
  • 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.

Miscellaneous

Medicolegal Pitfalls

  • Medicolegal pitfalls in Weber-Christian disease may involve unusual complications or adverse effects of drug therapy.

Special Concerns

  • Abrupt discontinuation of steroids may precipitate an adrenal crisis.
  • Medication toxicity may result from drug interactions (ie, cyclosporine with azathioprine) or from liver or kidney impairment.
 
Acknowledgments

The authors and editors of eMedicine gratefully acknolwedge the contributions of previous authors Oren Lifshitz, MD, and Heather Klein, MD, to the writing and development of this article.



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

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  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].

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  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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