Cutaneous hemophagocytosis: Clinicopathologic features of 21 cases

Abstract

Hemophagocytosis is well known in cytotoxic cutaneous T-cell lymphomas (CTCLs), in which it may represent a sign of hemophagocytic lymphohistiocytosis syndrome (HLHS), and is also typical of cutaneous Rosai-Dorfman disease (cRDD) (without prognostic relevance). Only rarely, has cutaneous hemophagocytosis (CH) been described in other skin conditions. To characterize the clinicopathologic features of CH in skin biopsy specimens from patients with conditions other than CTCL or cRDD. Case series analyzing clinicopathologic features and follow-up data on patients presenting with histopathologic signs of CH. Biopsy specimens from 21 patients were included. None of the patients had HLHS. The majority (n=11) presented with leukocytoclastic vasculitis. Other associated diseases were lupus erythematous (n=2), arthropod bite reaction (n=2), erysipelas (n=1), acne conglobata (n=1), and Sweet syndrome (n=1). Three patients had a nonspecific rash concomitant with Chlamydia pneumonia, middle ear infection, and pharyngitis, respectively. This was a single-center, retrospective study. Isolated CH in conditions other than CTCL and cRDD is a histopathologic finding related mostly to leukocytoclastic vasculitis. Extensive investigations should be performed only if patients have other signs or symptoms of HLHS.