Weber-Christian Disease

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

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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