Fulminant and accelerated presentation of dermatomyositis in two previously healthy young adult males: a potential role for endotheliotropic viral infection.

BACKGROUND: Dermatomyositis (DM) is a prototypic autoimmune syndrome, whereby immune-based microvascular injury is critical in the pathogenesis of skin lesions and the myopathy. Although not widely recognized or accepted as a pathogenetic trigger, endotheliotropic viral triggers including parvovirus B19 and cytomegalovirus have been linked to DM. At times, the clinical manifestations in DM can be fulminant with acute renal failure because of rhabdomyolysis, respiratory failure and gastrointestinal infarcts.

METHODS: Skin and lung tissues were processed for hematoxylin and eosin, immunohistochemical, immunofluorescent and reverse transcriptase in situ polymerase chain reaction studies.

CASE PRESENTATION: We present two cases of fatal DM in previously healthy immunocompetent males. One case had fatal catastrophic respiratory failure from diffuse alveolar damage; herpes simplex virus-2 RNA was uncovered in lung and skin biopsies during autopsy in the absence of classic cytopathic changes of herpes virus infection. The other case showed a Degos-like syndrome; parvovirus B19 RNA transcripts were found in cutaneous endothelium of affected skin.

CONCLUSION: An accelerated clinical course can occur in the setting of DM in previously healthy patients. A viral-based etiology should be explored because antiviral therapy may define a critical and potentially lifesaving therapeutic endeavor.