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Weber-Christian Disease Clinical Presentation

  • Author: Eyal Muscal, MD, MS; Chief Editor: Lawrence K Jung, MD  more...
 
Updated: Jan 22, 2015
 

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.

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Physical

Physical examination reveals erythematous, edematous, and tender subcutaneous nodules. See the images below.

Lesion of erythema nodosum: tender, erythematous, Lesion of erythema nodosum: tender, erythematous, nodular lesions located over the extensor surfaces of the legs.
Lesion of Weber-Christian disease: tender, erythemLesion 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.[11, 12]

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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.[13]

Similarities between Weber-Christian disease and alpha1-antitrypsin deficiency suggest that an altered regulation of a normal inflammatory process may be involved.[4, 14] Responses to cyclosporine support a T-cell mediated inflammatory or autoinflammatory process.[15]

Infections and postviral infectious responses have been explored, and no connection has yet been established.[7, 16]

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Contributor Information and Disclosures
Author

Eyal Muscal, MD, MS Assistant Professor, Section of Pediatric Immunology, Allergy, and Rheumatology, Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital

Eyal Muscal, MD, MS is a member of the following medical societies: Alpha Omega Alpha, American College of Rheumatology

Disclosure: Nothing to disclose.

Coauthor(s)

Eileen R Giardino, RN, MSN, PhD FNP-BC, ANP-BC, Associate Professor of Nursing, Department of Family Nursing, University of Texas Health Sciences Center Houston, School of Nursing

Eileen R Giardino, RN, MSN, PhD is a member of the following medical societies: American College Health Association, American Professional Society on the Abuse of Children, American Association of Nurse Practitioners, American Nurses Association, International Society for the Prevention of Child Abuse and Neglect

Disclosure: Nothing to disclose.

Robert W Warren, MD, PhD, MPH Chief Medical Information Officer, Professor, Department of Pediatrics, Division of Rheumatology, Medical University of South Carolina

Robert W Warren, MD, PhD, MPH is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American Association of Immunologists, American College of Rheumatology, Texas Pediatric Society, Childhood Arthritis and Rheumatology Research Alliance, Association of Medical Directors of Information Systems

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Thomas JA Lehman, MD FAAP, FACR, Clinical Professor of Pediatrics, Department of Pediatrics, Division of Pediatric Rheumatology, Weill Cornell Medical College; Chief, Hospital for Special Surgery

Thomas JA Lehman, MD is a member of the following medical societies: PM American Allergy Society

Disclosure: Nothing to disclose.

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, New York Academy of Sciences

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknolwedge the contributions of previous authors Moise Levy, MD, Oren Lifshitz, MD, Heather Klein, MD, and Angelo P Giardino, MD PhD MPH, to the writing and development of this article.

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

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

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

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

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

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

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

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

  9. Mavrikakis I, Georgiadis T, Fragiadaki K, Sfikakis PP. Orbital lobular panniculitis in Weber-Christian disease: sustained response to anti-TNF treatment and review of the literature. Surv Ophthalmol. 2010 Nov-Dec. 55(6):584-9. [Medline].

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

  11. Verrilli S, Ciarnella A, Laganà B, Calafiore S, Guglielmelli F, Basile L, et al. Ocular Inflammation: Can It Be a Sign of Activity of Weber-Christian Disease? A Case Report and Review of Literature. Ocul Immunol Inflamm. 2014 Oct 17. 1-4. [Medline].

  12. Mavrikakis I, Georgiadis T, Fragiadaki K, Sfikakis PP. Orbital lobular panniculitis in Weber-Christian disease: sustained response to anti-TNF treatment and review of the literature. Surv Ophthalmol. 2010 Nov-Dec. 55(6):584-9. [Medline].

  13. Requena L, Sanchez Yus E. Panniculitis. Part II. Mostly lobular panniculitis. J Am Acad Dermatol. 2001 Sep. 45(3):325-61; quiz 362-4. [Medline].

  14. Geraminejad P, DeBloom JR 2nd, Walling HW, Sontheimer RD, VanBeek M. Alpha-1-antitrypsin associated panniculitis: the MS variant. J Am Acad Dermatol. 2004 Oct. 51(4):645-55. [Medline].

  15. Pongratz G, Ehrenstein B, Hartung W, Scholmerich J, Fleck M. A patient with Pfeifer-Weber-Christian disease--successful therapy with cyclosporin A: case report. BMC Musculoskelet Disord. 2010. 11:18. [Medline].

  16. Douvoyiannis M, Litman N, Dulau A, Ilowite NT. Panniculitis, infection, and dermatomyositis: case and literature review. Clin Rheumatol. 2009 Jun. 28 Suppl 1:S57-63. [Medline].

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

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

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

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

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

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

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

  24. Moraes AJ, Soares PM, Zapata AL, Lotito AP, Sallum AM, Silva CA. Panniculitis in childhood and adolescence. Pediatr Int. 2006 Feb. 48(1):48-53. [Medline].

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

  26. Nahar N, Pardo V, Sadhu S, Young L, Gonzalez N, Jaimes EA. A case of Weber-Christian disease with collapsing glomerulopathy. Am J Kidney Dis. 2006 Sep. 48(3):484-8. [Medline].

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

  28. Spalding SJ, Meza MP, Ranganathan S, Hirsch R. Staphylococcus aureus panniculitis complicating juvenile dermatomyositis. Pediatrics. 2007 Feb. 119(2):e528-30. [Medline].

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

  30. Wouters CH, Martin TM, Stichweh D, et al. Infantile onset panniculitis with uveitis and systemic granulomatosis: a new clinicopathologic entity. J Pediatr. 2007 Dec. 151(6):707-9. [Medline].

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Lesion of erythema nodosum: tender, erythematous, nodular lesions located over the extensor surfaces of the legs.
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 focal opacity in the left lower lobe that looks like a pneumonia. This is a case of primary tuberculosis.
Lesion of Weber-Christian disease: tender, erythematous, nodular lesions located over the limbs with cutaneous atrophy.
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).
Magnification of previous specimen X 100.
Magnification of previous specimen X 200.
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).
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.)
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.)
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).
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).
 
 
 
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