Initial amyopathische Dermatomyositis mit Lungenbeteiligung und Anti-MDA5-Antikörpern

Dermatomyositis is a chronic inflammatory disorder with characteristic skin findings and a variable degree of muscle involvement. Some patients present with typical cutaneous manifestations but have subclinical (hypomyopathic) or absent (amyopathic) muscle disease. In these cases the diagnosis can be delayed, which might have a negative effect on the patient outcome. Determination of myositis-specific autoantibodies may help in this clinical situation. Anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive clinically amyopathic dermatomyositis is frequently associated with rapidly progressive interstitial lung disease. We report a case of a 56-year old woman with initially no dermatomyositis-typical skin or laboratory findings, where the correlation of clinical picture, dermatopathology and the finding of anti-MDA5 antibodies finally lead to the diagnosis of amyopathic dermatomyositis with lung involvement.