Korean J Med.  2017 Dec;92(6):552-557. 10.3904/kjm.2017.92.6.552.

A Case of Dermatomyositis in a Patient with a Neuroendocrine Tumor at the Ampulla of Vater

• Park JE
• Kang T
• Yoo JS
• Lee HS

• KMID: 2400922
• DOI: http://doi.org/10.3904/kjm.2017.92.6.552

Abstract

Dermatomyositis is an autoimmune disease characterized by chronic muscle inflammation that results in specific dermatological signs and decreased muscle strength. It is known to have a strong association with malignancy. Most neuroendocrine tumors arise from the gastrointestinal tract, with less than 1% of cases occurring at the ampulla of Vater. While cases of dermatomyositis associated with neuroendocrine tumors have been reported internationally, to date there have been no cases reported in Korea. This case report presents a 33-year-old male who had undergone pylorus-preserving pancreaticoduodenectomy for a neuroendocrine tumor located at the ampulla of Vater, and had been followed at the hospital after his procedure. Three years post-surgery, the patient developed serious muscle weakness in his upper-right and lower-left extremities, and was subsequently diagnosed with dermatomyositis. This paper presents the first domestic case of dermatomyositis developing in the context of a neuroendocrine tumor at the ampulla of Vater. A literature review on this subject follows in the discussion.

Keyword

Dermatomyositis; Autoimmune diseases; Malignancy; Neuroendocrine tumor; Ampulla of vater

MeSH Terms

Adult
Ampulla of Vater*
Autoimmune Diseases
Dermatomyositis*
Extremities
Gastrointestinal Tract
Humans
Inflammation
Korea
Male
Muscle Strength
Muscle Weakness
Neuroendocrine Tumors*
Pancreaticoduodenectomy