Table 1.

Clinical Variants of Dermatomyositis and their Associated Autoantibodies

Antibody	Cutaneous manifestations	Muscle manifestations	Pulmonary manifestations	Additional notes
Anti-Jo-138–41 (Also considered myositis specific auto-antibody)	• Mechanic’s hands	• Proximal muscle weakness	• ILD	• Anti-Jo1 in the setting of inflammatory arthritis, inflammatory myositis, mechanic’s hands, and ILD is known as “anti-synthetase syndrome”
				• Fever and Raynaud’s phenomenon may be additional features
Additional Anti-synthetase antibodies (PL-7, Pl-12, EJ, OJ, KS, ZO, YRS/HA)3,42	• Mechanic’s hands	• May or may not have muscle involvement	• ILD	• Also associated with anti-synthetase syndrome, as noted above
Anti-Mi-243,44	• V-sign	• Proximal muscle weakness	• Typically spares lungs	• Lower risk of malignancy compared to other dermatomyositis patients
	• Shawl sign
	• Gottron’s papules
	• Heliotrope rash
				• Steroid responsive45
Anti-p140/MJ/NXP-246	• Calcinosis leading to contractures	• Proximal muscle weakness	• High rates of ILD46	• Higher rates of cancer especially in male patients47,48
				• Younger age of onset
Anti-SAE49–51	• V-sign	• Myositis often follows cutaneous symptoms49,52,53	• Lower rates of ILD52	• Dysphagia52,53
	• Shawl sign		• If ILD present, tends to be mild and responsive to therapy53	• Systemic symptoms (fever, weight loss)52
	• Gottron’s papules
	• Heliotrope rash
	• Periungal erythema
Anti-TIF1-γ/p155-14054,55	• V-sign	• May or may not have muscle involvement	• Lower rates of ILD	• Associated with malignancy4,56–58
	• Shawl sign
	• Gottron’s papules
	• Hypopigmented patches with punctate telangiectasia or erythematous macules (“red on white” lesions)
Anti-MDA5/CADM1409	• Heliotrope rash	• Often clinically amyopathic	• High rate of ILD16	• Arthralgias, arthritis
	• Gottron’s papules		• Rapidly progressive disease	• Fever
	• Cutaneous ulceration			• Hoarseness, oral ulceration
	• Palmar papules			• Elevated serum ferritin8
	• Mucosal pain
	• Alopecia