Case Studies
The Telltale Rash
A Man with Pretibial Erythema
By Timothy Sielaff, M.D., Ph.D.
A hard-working rancher in his mid-60s casually showed a dermatologist friend a rash on his shin. The rash had been present for more than a month and though neither painful nor pruritic, it was weeping serous fluid and getting worse. The dermatologist noted a raised, nodular erythematous rash that occupied the entire left pretibial region. The dermatologist suspected necrotizing panniculitis and suggested the patient get a CT scan of the abdomen. A punch biopsy done the next day confirmed the diagnosis.
The man promptly saw his family physician. A thorough history and physical were unremarkable. Laboratory examination disclosed a lipase level over 15,000 U/L (normal <60 U/L). The CT scan showed an 8 cm mass in the body of the pancreas.
The patient was referred to our institution for further evaluation and treatment. Endoscopic ultrasound (EUS) showed the lesion and no evidence of lymph node metastases. Endoscopic ultrasound FNA confirmed the diagnosis. We performed a distal pancreatectomy and splenectomy for what proved to be an 8 cm acinar cell carcinoma with negative lymph nodes and margins.
Immediately after surgery, the rash subsided, and the patient’s blood lipase levels returned to normal. The patient’s hospital course was unremarkable.
The unifying findings in this case are the acinar cell cancer producing high levels of lipase, which resulted in the necrotizing panniculitis in the dependent portion of the left leg (that extremity had been traumatized in the distant past and had chronically poor venous drainage). Removal of the lipase-producing tumor allowed the rash to heal.
Pancreatic cancer is the 11th most common cancer in the United States, yet it is the fourth most common cause of death from cancer. An estimated 37,680 Americans will be diagnosed in 2008. The majority of these tumors are ductal adenocarcinomas; acinar cell carcinomas account for fewer than 1 percent of all pancreatic neoplasms. Acinar cell carcinomas arise from the digestive enzyme-producing cells of the pancreas, and in this patient, the serum lipase levels may prove to be a useful “tumor marker” to monitor for recurrence.
The occurrence of a rash with pancreatic tumors is rare. Glucagon-secreting islet cell tumors would be the most common pancreatic tumor associated with a rash. These rashes are typically necrolytic migratory erythemas and are associated with other digestive complaints such as diarrhea or abdominal cramping. Necrotizing panniculitis is reported to occur in a few systemic diseases including alpha-1 antitrypsin deficiency. Pancreatic etiologies are limited to a few case reports, but the recommendation is to rule out a pancreatic etiology if such as rash is unrelenting.
The case was discussed at the VPCI Multidisciplinary Tumor Conference and at a national Pancreatic Cancer Research Team teleconference. The VPCI and national opinions were discussed in concert with the patient’s local oncologist and adjuvant therapy was initiated in the patient’s hometown.
Thanks to the experience and insight of the patient’s dermatologist friend and his family physician, the tumor was diagnosed in a surgically treatable stage. Further systemic chemotherapy was expected to be beneficial. The patient is now back home getting the most appropriate care and doing the work he loves.
Timothy Sielaff is medical director of the Virginia Piper Cancer Institute in Minneapolis.