Definition of PanniculitisPanniculitis is an inflammation of the layer of subcutaneous fat underlying the epidermis of the skin. Acute panniculitis is also called nodular fat necrosis.[2]
Causes and Risk Factors of PanniculitisPanniculitis may result from a variety of conditions. While it is not possible to develop a firm classification of acute panniculitis, one may distinguish between panniculitis without systemic (throughout the body) disease and panniculitis with systemic disease. Panniculitis without systemic disease is usually due to trauma or a cold. One variant, subcutaneous fat necrosis of the newborn, may be due to a combination of obstetric trauma and hypothermia (low body temperature). Panniculitis with systemic disease includes collagen vascular disease (lupus and scleroderma), lymphoma, pancreatic cancer and pancreatitis. Symptoms of PanniculitisThe appearance of single or multiple crops of nodules in subcutaneous fat is the hallmark of acute panniculitis. The nodules are usually, but not always, tender. On occasion, they drain an oily solution and suppuration (pus) may occur. Individual lesions last from one to eight weeks before disappearing, and a pigmented depressed area may be left at the involved site. While some patients have only nodular panniculitis, which may or may not be relapsing, others may develop fever, abnormal liver function, involvement of the bone marrow, bleeding tendencies, nodular pulmonary lesions and evidence of pancreatic disease. This constellation of findings has been called Weber-Christian disease. Diagnosis of PanniculitisAcute panniculitis can be diagnosed only histologically, usually by biopsy of tissue. Once the lesion is identified, a search for the cause must be made. If systemic symptoms are present, the primary differential diagnosis is between collagen vascular disease, lympho-proliferative disorder and pancreatitis or pancreatic cancer. Treatment of PanniculitisTreatment is often unsatisfactory. Some physicians may use anti-inflammatory medications. Some patients with certain types of panniculitis may respond to combined chemotherapy with cyclophosphamide, bleomycin and prednisone. [2] How is Alpha-1 Antitrypsin Deficiency related to Panniculitus?Alpha-1 antitrypsin is the principal serum protease inhibitor. Severe deficiency of this important inhibitor is inherited as the ZZ phenotype, which occurs in about 1 in 2500 people.[3] In addition to the well-recongnized association with early-onset emphysema and cirrhosis, alpha 1-antitrypsin deficiency may be associated with panniculitis. Most of the cases with manifestations of cirrhosis and empysema have the severe type of deficiency (ZZ phenotype), whereas the partial deficiency (MZ phenotype) with less severe symptoms confined to cutaneous lesions, is more common. There are five possible mechanisms outlined for the pathogenesis of panniculitis due to the deficiency of alpha-1 antitrypsin.[4] Trauma is known to precipitate panniculitis in an individual with an inhibitor deficiency. In this case too, trauma during laproscopic pall bladder surgery in the periumbilical area could have precipitated the panniculitis. An early histopathological clue to the diagnosis of alpha-1 antitrypsin panniculitis is the splaying of neutrophils between collagen bundles in the reticular dermis.[5] In the present case too, in areas of early involvement, the same histopathological finding was present. Differentiating histopathological features, from other forms of panniculitis, are presence of large areas of normal fat adjacent to necrotic lobular and septa) areas, that contain intense polymorphonuclear and macrophage infiltration. Elastolysis is also characteristic of alpha-1 antitrypsin panniculitis.[6] These two features were also present in the case illustrated at http://www.ijdvl.com/default.asp [7] This case highlights the importance of testing for serum levels of alpha-1 antitrypsin in case of panniculitis, especially if the condition is chronic and indolent with spontaneous areas of ulceration and drainage of oily yellow fluid. Dapsone works effectively in most cases, as in the present case. Severe cases with life threatening complications like cirrhosis, emphysema and pleural effusions may require, in addition, oral steroids and alpha-1 proteinase inhibitor infusions.[4] No conclusion can be drawn, to recommend preoperative screening for alpha-1 antitrypsin deficiency in patients undergoing any abdominal surgery, laparoscopic or otherwise, based on this case. This will require establishing a more direct evidence of surgical trauma in precipitating alpha1antitrypsin panniculitis.
Questions To Ask Your Doctor About PanniculitisDo any more tests need to be done to diagnose the disease or the cause of the disease? What is the cause of panniculitis? What type of treatment will you be recommending? How effective is this treatment? Will you be
prescribing any medications?
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