Michele L Zerah1, Christine A DeWitt. 1. Department of Dermatology, Georgetown University Hospital/Washington Hospital Center, Washington, D.C., USA.
Abstract
BACKGROUND: Cutaneous findings associated with hemophagocytic lymphohistiocytosis (HLH) remain largely undescribed in the literature, yet are substantial and correlative with disease course. OBJECTIVE: To catalog the clinical findings of cutaneous eruptions associated with HLH. METHODS: We performed a retrospective chart review of patients meeting the criteria for HLH at two hospitals over 5 years. All patients meeting the criteria for HLH as defined by the HLH-2004 protocol were included. RESULTS: Cutaneous lesions were categorized based on clinical presentations and histology. Lesions independent of immunocompromised state were observed, including pyoderma gangrenosum, panniculitis, morbilliform eruptions, Stevens-Johnson syndrome, atypical targetoid lesions and bullae. Histologic findings were non-specific. CONCLUSION: Cutaneous eruptions as a consequence of HLH are variable in presentation and identified as a diagnosis of exclusion. Findings are both primarily and secondarily induced by altered immunity. Further study is needed to allow better understanding of the immunopathogenesis involved.
BACKGROUND: Cutaneous findings associated with hemophagocytic lymphohistiocytosis (HLH) remain largely undescribed in the literature, yet are substantial and correlative with disease course. OBJECTIVE: To catalog the clinical findings of cutaneous eruptions associated with HLH. METHODS: We performed a retrospective chart review of patients meeting the criteria for HLH at two hospitals over 5 years. All patients meeting the criteria for HLH as defined by the HLH-2004 protocol were included. RESULTS:Cutaneous lesions were categorized based on clinical presentations and histology. Lesions independent of immunocompromised state were observed, including pyoderma gangrenosum, panniculitis, morbilliform eruptions, Stevens-Johnson syndrome, atypical targetoid lesions and bullae. Histologic findings were non-specific. CONCLUSION:Cutaneous eruptions as a consequence of HLH are variable in presentation and identified as a diagnosis of exclusion. Findings are both primarily and secondarily induced by altered immunity. Further study is needed to allow better understanding of the immunopathogenesis involved.