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Weber-Christian Disease

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

Background

Panniculitis refers to a broad spectrum of diseases that involve inflammation of the subcutaneous fat layer of the skin. Weber-Christian disease is an eponym for a form of panniculitis, idiopathic nodular panniculitis, which is characterized by subcutaneous nodules, inflammatory cells in the fat lobules, and systemic symptoms.[1] The nomenclature of this and other related diseases is confusing, and some authors believe that the eponym should be abandoned and that more specific diagnoses should be made on the basis of pathogenesis, cause, or additional diagnostic testing. The history of the eponym began in 1892 when Pfeifer first described the skin condition now known as Weber-Christian disease, or idiopathic lobular panniculitis. In 1925, Weber further depicted the syndrome[2] , and Christian emphasized the significance of fever as part of the syndrome. The syndrome became known as Weber-Christian disease in 1928.[3, 4]

Increasing study and diagnostic sophistication have differentiated Weber-Christian disease from diseases such as lupus panniculitis, factitial panniculitis, panniculitis associated with pancreatic disease, histiocytic cytophagic panniculitis, and alpha1-antitrypsin deficiency panniculitis.[5] With further differentiation, additional diseases will be distinguished from Weber-Christian disease. A review of 30 Mayo Clinic patients diagnosed with Weber-Christian disease stated that: "Because separate and distinct forms of fat lesions have been described, we believe that the eponym should be abandoned and the more specific diagnoses should be made on the basis of pathogenesis or cause."[6] At this time, the eponym Weber-Christian disease still refers to cases of nodular panniculitis with systemic signs and symptoms that remain idiopathic.

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Pathophysiology

Weber-Christian disease is a classic skin condition that features recurring inflammation in the subcutaneous fat layer of the skin. The involved skin areas manifest as recurrent crops of erythematous, sometimes tender, edematous, subcutaneous nodules. Lesions distribution is symmetric, and the thighs and lower legs are affected most frequently. Malaise, fever, and arthralgias often occur. Nausea, vomiting, abdominal pain, weight loss, hepatomegaly, and additional systemic features may also occur. The key pathologic finding on microscopy is a nodular inflammatory pattern of the fat lobules. Because the etiology is unknown, Weber-Christian disease is often referred to as idiopathic lobular panniculitis.[7, 8]

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Epidemiology

Frequency

United States

Ambiguity surrounding Weber-Christian disease versus other closely related conditions makes it difficult to determine the frequency of the diagnosis. It is recognized as a rare condition in adults and even more rare in pediatrics. White and Winkelmann's 1998 case record review of Weber-Christian disease at the Mayo Clinic found only 30 cases in a 28 year span between 1960 and 1998. They observed that cases of primary panniculitis are rare and that physicians may see only one or a few cases in a lifetime.[6]

International

The incidence and prevalence of Weber-Christian disease is unknown both in the United States and internationally. Weber-Christian disease is rare in adults and even rarer in children. For example, a chart review of children and adolescents in Brazil over a 20-year period (1983-2002) found 35 pediatric and adolescent cases of panniculitis, with only 6 cases meeting criteria for Weber-Christian disease.

Mortality/Morbidity

Weber-Christian disease is a more serious form of panniculitis because of its systemic manifestations. The disease course varies, and prognosis depends on which organs are affected, the severity of organ involvement, and the response to therapy.

Weber-Christian disease may involve the lungs, heart, intestines, spleen, kidney, adrenal glands, and even orbits.[9] Significant morbidity and mortality may occur in patients with inflammation involving visceral organs.

The clinical course in patients with only cutaneous manifestations may be characterized by exacerbations and remissions of the cutaneous lesions for several years before the disorder subsides.

Race

No racial predilection is reported.

Sex

Weber-Christian disease occurs more often in women, who comprise approximately 75% of reported cases. The disease is rare in the pediatric population, with a slight predilection for females over males. In the Moraes and colleagues Brazilian study, 4 of the 6 cases of Weber-Christian disease were girls.

Age

Weber-Christian disease may occur in children but is rare. It has been reported most frequently in people in the fourth to seventh decades of life, and 75% of cases occur in women after the second decade of life.[10]

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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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