ABSTRACT
BACKGROUND: Hydroa vacciniforme (HV) is a disease of unknown origin characterized by erythema, vesicles, necrosis, and varicelliform scars in light-exposed skin. Systemic involvement is absent. A few patients have been reported with "severe HV" with systemic involvement, development of non-Hodgkin's lymphoma, and a poor prognosis. OBJECTIVE: Our purpose was to characterize and differentiate our patients' disease from HV. METHODS: We performed a retrospective clinicopathologic study of 14 children previously diagnosed as having "severe HV." RESULTS: The extension and severity of the cutaneous lesions, fever, wasting, failure to thrive, hepatosplenomegaly, vasculitis, panniculitis, and potential development of lymphoma are features that clearly differentiate edematous scarring vasculitic panniculitis from HV. CONCLUSION: Edematous scarring vasculitic panniculitis is a novel multisystemic disease with malignant potential that is not related to classic HV.
