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Panniculitis

Panniculitis is the term used to describe diseases where the major focus of inflammation is in the subcutaneous tissue. In general, panniculitis presents as erythematous or violaceous nodule in the subcutaneous fat that may be tender or not, that may ulcerate or heal without scarring, and that may be soft or hard on palpation. Thus, the term panniculitis describes a wide spectrum of disease manifestations, although diagnostic clues can be derived from the history, distribution, or characteristics of the lesions. An accurate diagnosis requires an ample deep skin biopsy that should reach down to or even beyond the fascia.

The panniculitides are classified histologically as lobular or septal depending on where the disease process begins. Panniculitis may also be associated with vasculitis or in most cases without vasculitis.

Only idiopathic lobular panniculitis (Pfeiffer­Weber-Christian disease), pancreatic panniculitis, and al antitrypsin-deficiency panniculitis are briefly discussed here. Other diseases in which panniculitis occurs are referred to in the table, and the reader is also referred to Fitzpatrick's Dermatology in General Medicine, 5th ed.1

Idiopathic lobular panniculitis, which occurs predominantly in females 30 to 60 years of age, manifests as subcutaneous inflammatory nodules, primarily on the lower extremities but also on the trunk and elsewhere, that erupt in crops and are usually tender. New waves of lesions appear at intervals. Occasionally, lesions can break down, discharging an oily yellow-brown liquid; and these inflammatory nodules are generally accompanied by malaise, fatigue, fever, arthralgia, and myalgia. Due to systemic involvement there may be focal necrosis in the intravisceral and perivisceral fat of internal organs, including the mesenteric and omental fat, pericardium, and pleura. Organ involvement may present as hepatomegaly, abdominal pain, nausea, and vomiting.

Leukocytosis and an elevated erythrocyte sedimentation rate are further characteristics of this disease, of which the etiology is unknown. The course and prognosis are variable; the prognosis is good in patients who have only cutaneous involvement, but lobular panniculitis associated with prominent visceral involvement may lead to death. There is no uniform effective therapy recognized; fibrinolytic agents, chloroquine, azathioprine, thalidomide, cyclophospharnide, and cyclosporine have been tried.

α 1 antitrypsin-deficiency panniculitis is also characterized by recurrent tender, erythematous, subcutaneous, ulcerating nodules ranging from 1 to 5 cm and located predominantly the trunk and the proximal extremities. Nodules break down and discharge a clear serous or oily fluid. Diagnosis is substantiated by a de, in the level of serum α 1 antitrypsin, and treatment consists of oral dapsone in doses up to 200 mg/d. The intravenous infusion of human α 1 proteinase inhibitor concentrate has been sit to be very effective.

Pancreatic panniculitis is characterized clinically by painful erythematous nodules that occur at any site. It is frequently accompanied by arthritis and polyserositis and is associated with either pancreatitis or pancreatic carcinoma. This form of panniculitis affects middle-age to elderly individuals, males more often than females. The history usually reveals alcoholism abdominal pain, weight loss, or recent-onset diabetes mellitus. Skin lesions are tender, warm, erythematous nodules and plaques that fluctuate and occur at any site with a predilection for legs, buttocks, and abdomen. Skin biopsy reveals lobular panniculitis, and after biopsy of a lesion, liquefied fat drains from the biopsy site. General examination may reveal pleural effusion, ascites and arthritis, particularly of the ankles.

Laboratory examinations show eosinophilia, hyperlipasemia, hyperamylasemia, and increased excretion of amylase and/or lipase in the urine. The pathophysiology is probably a break down of subcutaneous fat caused by enzymes (amylase, trypsin, lipase) released into the circulation from a diseased pancreas. The course and prognosis depend on the type of pancreatic disease.

Treatment 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.