A 54-year-old female with recently diagnosed diabetes mellitus presents to the office with a skin rash. She states that the rash started three weeks ago on her forearm as a small, elevated red area. It gradually involved the surrounding skin, and became an erythematous, itchy, painful rash that did not respond to topical 1% hydrocortisone. Approximately one week ago, the rash started clearing up from the center. The patient has not been feeling well for the past 1-2 months. She also complains of lethargy, weight loss, diarrhea, abdominal cramps, and occasional facial flushing. On physical examination, a 4 X 5 cm, erythematous, scaly rash, which appears to be clearing from center, is noted. The borders appear slightly elevated and crusty. Angular cheilosis is also noted. What is the most likely diagnosis of this patient?

Question

A 54-year-old female with recently diagnosed diabetes mellitus presents to the office with a skin rash.  She states that the rash started three weeks ago on her forearm as a small, elevated red area.  It gradually  involved the surrounding skin, and became an erythematous, itchy, painful rash that did not respond to topical 1% hydrocortisone.  Approximately one week ago, the rash started clearing up from the center.  The patient has not been feeling well for the past 1-2 months.  She also complains of lethargy, weight loss, diarrhea, abdominal cramps, and occasional facial flushing.  On physical examination, a 4 X 5 cm, erythematous, scaly rash, which appears to be clearing from center, is noted.  The borders appear slightly elevated and crusty.  Angular cheilosis is also noted.  What is the most likely diagnosis of this patient?

Quizplus · Accepted Answer

Explanation: The patient has the characteristic rash of glucagonoma, which is also known as "necrolytic migratory erythema". Glucagonoma is a rare pancreatic tumor associated with mild diabetes and a classic skin rash. Most glucagonomas are malignant and have metastasis, mainly in the liver, at the time of diagnosis. Some of its clinical manifestations are due to the secretion of other peptides, such as VIP, calcitonin, and GLP1. The diagnosis is suspected clinically, and then confirmed by measuring glucagon levels, which are generally very high in these patients. The primary treatment is surgical. (Choices A and B) The rash described is not characteristic of SLE or pellagra. Pellagra is due to niacin deficiency, and is characterized by dementia, diarrhea, dermatitis, stomatitis and cheilosis. The associated dermatitis or skin rash is symmetrically distributed in sun-exposed areas, and often forms vesicles and blisters. (Choice C) A fungal skin infection does not produce systemic manifestations, unless the rash gets bacterial superinfection. (Choice E) The skin rash in systemic mastocytosis is urticarial; however, maculopapular, vesicular or pustular rashes have been reported, particularly in children. The rash is more often generalized. Rubbing the edge of the rash produces wheals and erythema; this is called "Darier's sign". About 50% of these patients also have hepatosplenomegaly. Educational Objective: Glucagonoma is a rare pancreatic tumor associated with mild diabetes and a classic skin rash.

A 54-Year-Old Female with Recently Diagnosed Diabetes Mellitus Presents to the Office