Diagnosis of amyopathic dermatomyositis after two intrauterine fetal deaths

Abstract

Chronic histiocytic intervillositis (CHIV) is an uncommon condition, characterized by an infiltrate of mononuclear cells of maternal origin in the intervillous space that has been related to placenta insufficiency and poor perinatal outcomes. The aetiology is unclear, although maternal immunological aggression toward fetal tissues has been proposed. Dermatomyositis (DM) is a multisystem autoimmune inflammatory myopathy. Different autoantibodies have been associated with particular clinical phenotypes; presence of anti-melanoma differentiation-associated gen 5 (MDA5) antibody has been associated with rapidly progressive interstitial lung disease and severe skin lesions, none of which the woman had. Described here is a case of a woman diagnosed with amyopathic DM with positive anti-MDA5 antibodies after two intrauterine fetal deaths. Pathological examination of the placenta in both pregnancies showed CHIV. The presence of a potential relationship between both processes is discussed.