A 50–year–old man presented to his dermatologist with a 2–week history of a rash over his extremities. The nodules were painful, raised and violaceous (Fig. 1). He was empirically treated with oral Prednisone for a presumed diagnosis of erythema nodosum but his symptoms did not resolve. Skin biopsy was done and was inconclusive. Over the following 2 weeks his rash worsened. Given the lack of resolution of his symptoms, the Prednisone was discontinued and Plaquenil was started out of concern for vasculitis and he was referred to the hospital for admission. Repeat biopsy demonstrated subcutaneous fat necrosis consistent with panniculitis. Serum amylase was 2192 and lipase was 7360. Transaminases and triglycerides were normal. He was placed on bowel rest and his amylase and lipase peaked at 8410 and 13,720 and then gradually trended down over the next few weeks. The patient never had abdominal pain. CT scan of his abdomen did not reveal any pancreatic pathology or inflammation. Discussion: Pancreatitis is a common disease with more than 80,000 cases annually in the United States. It usually presents with abdominal pain. Panniculitis is a very uncommon presentation of pancreatitis with estimates of the incidence at one case per ∼900 episodes of pancreatitis. Panniculitis is characterized by diffuse subcutaneous fat necrosis involving the extremities and trunk and is caused by high levels of circulating pancreatic enzymes. The skin manifestations can occur at any time in the course of the disease but typically precede the diagnosis of pancreatitis when abdominal pain is not present. The skin biopsy (Fig. 2) demonstrates the major histological findings. The arrow in Figure 2A indicates an area of fat necrosis. The arrow in Figure 2B shows an area of inflammatory infiltrate. In Figure 2C, the upper arrow indicates an inflammatory infiltrate above a region of fat necrosis with ghost like outlines of coalescing adipocytes with saponification (lower arrow). The arrow in Figure 2D shows calcium deposition. These features, particularly the saponification and calcium deposition, are consistent with pancreatic fat necrosis.
We report this case to increase awareness of this syndrome as pancreatic panniculitis has clinical features that can easily mimic other entities like erythema nodosum or vasculitis. The lack of abdominal symptoms often leads to a delay in diagnosis that can contribute to mortality rates approaching 25%. Treatment is largely supportive and directed at the underlying cause of pancreatitis.
Figure 1Cutaneous manifestations.
Figure 2Histology from biopsy specimen.