Dermatomyositis

 

Dermatomyositis is characterized by inflammatory myositis (proximal muscle weakness) and skin disease–Edema and pinkish violet discoloration around the eyelids and cheeks is called a Heliotrope eruption;  Flat-topped, polygonal, violaceous papules over the knuckles are referred to as Gottron’s papules; less commonly hyperkeratosis, scaling, fissuring and hyperpigmentation over the fingertips, sides of the thumb and fingers.  Telangiectatic vessels often become prominent in the proximal nailfolds of the hands.  Associated erythema and scale may occur over the shoulder girdle, arms and neck and is referred to as “shawl sign.”

Patients often have elevated CPK enzymes or aldolase levels , positive anti-Jo-1 antibody titers, elevated CRP (C reactive protein) and nondestructive arthritis or arthralgias.  Myogenic changes are noted on EMG (short-duration, polyphasic motor unit potentials with spontaneous fibrillation potentials).

Treatment:  Systemic steroids are often used for acute onset.  For chronic disease a variety other therapeutic considerations include azathioprine, IVIG, etanercept, infliximab, cyclophosphamide and leflunomide have been used with some success.

Dermatomyositis is not prevalent to Las Vegas or Henderson but we do see it locally.

To read more about Dermatomyositis click HERE.