Idiopathic Lobular Panniculitis

Idiopathic Lobular Panniculitis

Article
Digestive Health
Healthy Lungs
+4
Contributed byLester Fahrner, MD+1 moreJul 25, 2022

What are the other Names for this Condition? (Also known as/Synonyms)

  • Weber-Christian Disease
  • Pfeiffer-Weber-Christian Disease

What is Idiopathic Lobular Panniculitis? (Definition/ Background Information)

  • Idiopathic Lobular Panniculitis is condition in which inflammation occurs in the subcutaneous tissue, just beneath the skin. The term ‘panniculitis’ indicates inflammation of fat tissue
  • The condition causes the presence of painful or painless subcutaneous nodules all over the body; the skin over these nodules may be red in color. The cause of the condition is unknown (idiopathic meaning of unknown cause) 
  • Idiopathic Lobular Panniculitis may also have the involvement of many vital organs such as the heart, lungs, kidneys, intestines, and spleen
  • Complications may arise from the nodules that include ulceration and bleeding. This can cause scar formation on healing
  • The treatment involves the use of medications and steroids; however, the prognosis depends upon many factors including the severity of the signs and symptoms and the organs that are affected

Who gets Idiopathic Lobular Panniculitis? (Age and Sex Distribution)

  • Idiopathic Lobular Panniculitis is a rare condition that is predominantly observed in women in the 30-60 years age group. Occasionally, children may also be affected
  • No racial or ethnic group predilection is observed and the condition is observed worldwide

What are the Risk Factors for Idiopathic Lobular Panniculitis? (Predisposing Factors)

  • No specific risk factors have been associated with Idiopathic Lobular Panniculitis

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Idiopathic Lobular Panniculitis? (Etiology)

  • The exact cause of Idiopathic Lobular Panniculitis is currently unknown
  • Research is currently on to identify the relevant causative factors
  • The condition is not caused by sun-exposure or due to trauma to the skin

What are the Signs and Symptoms of Idiopathic Lobular Panniculitis?

The signs and symptoms of Idiopathic Lobular Panniculitis depend upon the part of the body that is affected.

  • The nodules are mostly observed in the thighs and lower legs. The nodules appear in a symmetric pattern and may present with or without any pain
  • The subcutaneous nodules may be present on other parts of the body too, such as the chest and back
  • The skin lesions can have a brown discharge
  • Fever
  • Tiredness
  • Joint and muscle pain
  • Weight loss

Occasional involvement of other organs such as the following:

  • Pericardium (covering of heart)
  • Pleura (covering of lung), with chest pain and breathing difficulties
  • Fat around the liver and intestines: When this occurs the signs and symptoms may include nausea and vomiting, abdominal pain, enlarged liver (hepatomegaly)

How is Idiopathic Lobular Panniculitis Diagnosed?

Idiopathic Lobular Panniculitis may be diagnosed using the following tools:

  • Complete physical examination with comprehensive evaluation of medical history
  • Blood tests:
    • To detect serum amylase and lipase levels
    • Complete blood count (CBC) with differentials may show low white cell count and decreased hemoglobin (anemia)
    • Blood electrolyte may reveal abnormal levels
    • Erythrocyte sedimentation rate (ESR) blood test may show increased ESR
    • C-reactive protein blood test may show increased C-reactive protein levels
    • May reveal abnormal liver function test
  • Blood and tissue culture for bacteria, fungal, and viral infections
  • Urine tests to check for lipase and amylase levels
  • Radiographic studies of the affected region
  • Deep skin biopsy: A biopsy is performed and sent to a laboratory for a pathological examination. The specimen must include epidermis, dermis, and a substantial amount of subcutaneous fat. The pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis

Depending upon the organ involved, the healthcare provider may undertake further studies such as:

  • Echocardiogram (for the heart)
  • Chest X-ray, CT, or MRI scans of the affected region
  • Abdominal ultrasound
  • Liver function test

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Idiopathic Lobular Panniculitis?

The complications due to Idiopathic Lobular Panniculitis may include:

  • The nodules may ulcerate and bleed, resulting in the formation of scars on healing
  • Bacterial and fungal infections: The condition may provide a suitable environment for bacteria and fungi to grow and thrive resulting in secondary infections
  • The lesions can last for years resulting in self-image issues, emotional stress, and psychological trauma in some individuals
  • If the emotional stress is severe, it may even lead to depression and social isolation

How is Idiopathic Lobular Panniculitis Treated?

The treatment of Idiopathic Lobular Panniculitis may involve the following measures:

  • Systemic steroids to control severity of the condition
  • Fibrinolytic agents (which are used to prevent the clotting of blood)
  • If the signs and symptoms are severe, then admission to a hospital may be necessary
  • Medications including antibiotics and antifungals to treat infections, in case of secondary infections
  • Providing reassurance and help with feelings of stress and anxiety
  • Follow-up care with regular screening and check-ups are important, since the condition can last for many months and years

How can Idiopathic Lobular Panniculitis be Prevented?

Current medical research has not established a method of preventing Idiopathic Lobular Panniculitis. However, the severity of the condition may be controlled through continuous medical support and treatment.

What is the Prognosis of Idiopathic Lobular Panniculitis? (Outcomes/Resolutions)

  • The prognosis of Idiopathic Lobular Panniculitis depends upon the severity of the signs and symptoms and involvement of the vital organs of the body. It also depends upon the underlying condition
  • Idiopathic Lobular Panniculitis can be a fatal condition if severe involvement of the heart, lungs, kidneys, or liver occurs

Additional and Relevant Useful Information for Idiopathic Lobular Panniculitis:

  • Idiopathic Lobular Panniculitis is also known as Weber-Christian Disease, after the scientists who studied and made well known the features of this disorder
  • There are many different types of panniculitis, and it has been difficult to obtain an accurate picture of their true incidence since they may be associated with so many conditions. The different types may include:
    • Lupus panniculitis
    • Pancreatitis-associated panniculitis
    • Alpha-1 antitrypsin deficiency associated panniculitis
    • Infective panniculitis
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On the Article

Krish Tangella MD, MBA picture
Approved by

Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team
Lester Fahrner, MD picture
Reviewed by

Lester Fahrner, MD

Chief Medical Officer, DoveMed Team

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