The patient is a 19 year old female who presents with proximal muscle weakness, a rash on her eyes, chest, and hands, and dysphagia. She describes difficulty getting out of a chair, doing her hair, and climbing stairs. She feels that the food gets stuck in her chest when she swallows both liquids and solids. These symptoms have been present for 2 months.

Physical exam shows a purplish discoloration of the eyelids, a maculopapular rash on the chest, a scaly rash over the knuckles, and proximal muscle weakness in the arms and the legs.There are hard palpable nodules around the knees and hands.

Laboratory testing showed an elevated CPK of 8,000.

Slide one: Heliotrope rash: purplish discoloration of the upper lids.

Slide two: Maculopapular rash on the V-distribution of the neck and chest

Slide three: Gottron’s papules: a scaly rash over the knuckles

Slide four: Calcinosis at the knees

Muscle biopsy showed inflammatory infiltrate around the muscle fibers.

Slide five: Mononuclear inflammation in around muscle fibers.

Xray showed subcutaneous calcification around the knees.

Slide six

Discussion: Polymyositis/dermatomyositis(PM/DM) is a rare autoimmune inflammatory disorder resulting in muscle tissue destruction with resultant muscle weakness. Age of onset has 2 peaks: in young individuals (5-15 yrs) and middle aged persons (45-55 yrs) Proximal muscle groups are affected resulting in a proximal myopathy. This affects activities such as getting out of a chair, stair climbing, and overhead or reaching activities. The heliotrope rash and Grotton’s papules are specific for dermatomyositis. The maculopapular rash in the V-distribution of the chest is not specific for dermatomyositis. Subcutaneous calcinosis is seen in the juvenile form of PM/DM. In the juvenile form of PM/DM vasculitis of the GI tract can also be seen. Involvement of the smooth muscle of the upper GI tract can cause dysphagia. In severe cases, pulmonary fibrosis and pericarditis/myocarditis can occur. Diagnosis is made by muscle biopsy. Treatment consists of high dose corticosteroids and methotrexate.