A chronic disorder characterized by relapsing febrile
episodes and panniculitis. Systemic manifestations as a result of visceral
involvement may be present.
Lipophagic Panniculitis; Nodular non-Suppurative
Panniculitis; Pfeiffer-Weber-Christian Syndrome.
This recurring inflammation in the fat layer of the skin
was first described by Victor Pfeiffer, German physician in 1892.
Unknown. Some authors think that some of these patients
were misdiagnosed for other diseases, for example, for subcutaneous T-cell
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,
i.e., 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-Makay
It is characterized by recurrent febrile
episodes and 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
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).
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.
The administration of supplemental steroid
stress doses must be considered in patients receiving chronic steroid treatment.
Sweet Syndrome: A 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 to 20% of cases, with hematologic malignancy
Erythema Nodosum: Belongs to a group of skin disorders
characterized by painful nodules most often affecting the lower extremities.
It is often associated with recurring episodes of fever, malaise, fatigue,
joint pain and the mechanism remains unknown.
Ehman F, Harth M, Spouge AR: Christian disease with severe polyarthritis
and polyosteitis. J Rheumatol 29(5):1102, 2002.