Weber–Christian disease

Alternate names

Panniculitis nodular nonsuppurative; Weber-Christian panniculitis; Idiopathic nodular panniculitis; Relapsing febrile nodular nonsuppurative panniculitis; Pfeiffer-Weber-Christian syndrome

Definition

Nodular nonsuppurative panniculitis describes a rare group of skin disorders characterized by tender, painful bumps below the surface of the skin (subcutaneous nodules) that usually lead to inflammation of the subcutaneous layer of fat (panniculitis).

Cause

The exact cause of nodular nonsuppurative panniculitis is unknown.

Signs and symptoms

• This condition is characterized by tender, painful bumps below the surface of the skin (subcutaneous nodules) that usually lead to inflammation of the subcutaneous layer of fat (panniculitis).
• These nodules tend to be 1-2 centimeters in length and most often affect the legs and feet.
• In most people, this condition is associated with fever, a general feeling of ill health (malaise), muscle pain, and/or abdominal pain.
• These symptoms may subside after a few days or weeks and may recur weeks, months, or years later.

Clinical presentation

For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. 80%-99% of people have these symptoms

• Abdominal pain(Pain in stomach)
• Aplasia/Hypoplasia of the skin(Absent/small skin)
• Arthralgia(Joint pain)
• Edema(Fluid retention)
• Erythema
• Fever
• Myalgia(Muscle ache)
• Nausea and vomiting
• Panniculitis(Inflammation of fat tissue)
• Subcutaneous nodule(Firm lump under the skin)
• Weight loss

5%-29% of people have these symptoms

• Autoimmunity(Autoimmune disease)
• [[Hepatomegaly](Enlarged liver)
• Inflammatory abnormality of the eye
• Splenomegaly(Increased spleen size)

Treatment

Treatment for nodular nonsuppurative panniculitis (NNP) generally aims at controlling and relieving the symptoms that an individual has. Before treatment is initiated, a work-up should be completed to determine whether the condition is secondary to another underlying disorder. If there is an underlying disorder, treatment of this disorder may relieve the symptoms of NNP. In some cases, skin lesions heal spontaneously (remission) but the lesions often later return. There is no treatment method found to be effective for all individuals with NNP. Medications used to treat the condition may include systemic steroids (such as prednisone) to suppress sudden attacks; nonsteroidal anti-inflammatory drugs (NSAIDs) to reduce fever and other signs of malaise; and/or immunosuppressive drugs. Relief of symptoms in some affected individuals has also been reported with fibrinolytic agents (medications that help prevent blood clots), hydroxychloroquine, azathioprine, thalidomide, cyclophosphamide, tetracycline, cyclosporin, mycophenolate, and clofazimine.

Prognosis

• It is not possible to predict the prognosis for specific individuals (including when and if remission will occur), as the prognosis widely varies in individuals with the condition.
• Those with primarily cutaneous (skin) involvement may experience periods of exacerbations and remission of symptoms with minimal involvement of other organs for several years before the disorder resolves.
• The disease tends to recur at intervals of weeks or months.
• In individuals with severe involvement of the heart, lungs, intestines, spleen, kidney, or adrenal glands, morbidity and mortality are significant and these individuals may not survive.

External links

Classification	ICD-10: M35.6 ICD-9-CM: 729.30 MeSH: D010201 DiseasesDB: 14055
External resources	eMedicine: ped/2429

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