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At a glance

A chronic disorder characterized by relapsing febrile episodes and panniculitis. Systemic manifestations as a result of visceral involvement may be present.

Synonyms

Lipophagic Panniculitis; Nodular non-Suppurative Panniculitis; Pfeiffer-Weber-Christian Syndrome; Relapsing Febrile Nodular Nonsuppurative Panniculitis Syndrome.

History

This recurring inflammation in the fat layer of the skin was first described by Victor Pfeiffer, a German physician, in 1892.

Incidence

The exact incidence remains unknown. Some authors think that some of these patients were misdiagnosed for other diseases, for example, for subcutaneous T-cell lymphomas.

Diagnosis

The Diagnosis is made on the aspect of the skin and subcutaneous biopsy. Differential diagnosis with the other panniculitis must be made. Weber-Christian Disease (WCD) is usually a diagnosis by default, ie, being unable to establish an underlying etiology or pathogenesis. The most current misdiagnoses are lupus profundus, all the collagen vascular diseases: alpha-1 antitrypsin deficiency, pancreatic disease, generalized lipodystrophy, paraproteinemia with C-1 inhibitor deficiency, eosinophilic myalgia of fasciitis syndromes and in children, the rare Rothmann-Makai Syndrome.

Clinical aspects

It is characterized by recurrent febrile episodes associated with erythematosus subcutaneous nodules. Recurrent crops of symmetrical, tender lesions, 1 to 2 cm in size, may appear in any area. These nodules rarely suppurate, and spontaneous regression results in a hyperpigmented atrophic scar that is depressed consequent to subcutaneous fat necrosis. Weber-Christian disease implies systemic involvement when the skin lesions are accompanied by arthralgia, myalgias, and abdominal pain. In severe instances, the inflammation can involve the liver, lungs, myocardium, spleen, kidneys, and adrenal glands. Xanthogranuloma of the dura are described.

Precautions before anesthesia

A complete medical history of the severity of the illness must be up to date. Assess for involvement of kidneys, lung, and heart. Evaluate biology (cell blood count, creatinine, liver enzymes). Check for the possibility of congestive heart failure (echocardiography if necessary).

Anesthetic considerations

Xanthogranulomatous masses may appear everywhere within the body including the dura mater. The size of the infiltrates can be microscopic infiltration to 8 cm in diameter. The presence of these nodules should be considered when using a locoregional anesthesia.

Pharmacological implications

The administration of supplemental steroid stress doses must be considered in patients receiving chronic steroid treatment.

Other conditions to be considered

  • Sweet Syndrome: Rare skin disorder with an unknown genetic or acquired cause. It is characterized by fever, arthritis, and the sudden onset of a severe cutaneous rash. The rash consists of an asymmetric distribution of bluish-red, tender papules that usually occur on the arms, legs, face or neck. In approximately 80% of cases, it is idiopathic and spontaneous. This clinical manifestation can be associated, in 10 ...

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