Viral form of hemophagocytic syndrome with erythrodermal clinical picture--case report

Hemophagocytic syndrome is patophysiological entity with proliferation and over-activation of macrophages, with hemophagocytosis and production of proinflammatory cytokines. It arises as hereditary forms, or acquired, during viral, autoimmune or malignant diseases, and is usually a disorder with fulminant course and high incidence of lethal outcome. The precise mechanism is not resolved; it is a consequence of cytokine storm generated by over-activated T cells and macrophages, due to defects in T cellular cytototoxic function and inadequate down-regulation of immune response. A male patient, 26 years old, previously healthy, is presented. Generalized exfoliative dermatitis and lymphadenomegalia had lasted for half a year before admission to the hospital. Hemophagocytosis in lymph gland histology was diagnostic, with T cellular immunohistochemical profile CD3+, CD5-, CD8/-, CD43+/-, CD45RO+, bcl-2+, and numerous CD68+ histiocytes. Apart from elevated titer of Adenovirus serology, other laboratory findings and bone marrow histology were within normal limits. Two weeks of oral antibiotic and topical skin corticosteroid therapy were followed by a rapid improvement of clinical features. Residual skin lesions, linear petechia and flares of pale pink erythema used to recur for the next half a year. During the follow up, two years later there was no lymph gland enlargement, skin rash, or other signs. The diagnosis of virus-associated hemophagocytic syndrome with mild clinical course and seemingly spontaneous improvement was established, although it did not fulfill all proposed diagnostic criteria. It is possible that it increased the clinical awareness for these mild forms in immune-competent patients could account for the improved recognition of atypical cases with favorable outcome.