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Weber-Christian Disease Treatment & Management

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

Medical Care

No uniformly effective therapy for Weber-Christian disease is known. Clinical experience, especially in children and adolescents, has pointed to the value of corticosteroids and immunosuppressive agents.

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

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Surgical Care

No surgical treatment is indicated.

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Consultations

Consultation with a pediatric dermatologist will help in considering differential diagnoses and possible causes of panniculitis. The dermatologist may also perform a skin biopsy for pathological review.

Consultation with a pediatric rheumatologist and infectious disease specialist will help to determine a differential diagnosis and implement a treatment regimen.

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Diet

No specific dietary requirements are noted.

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Activity

Activity is ad lib, and trauma to the affected areas should be avoided.

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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
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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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