Track
Case ReportsAbstract
Hydroa vacciniforme-lymphoproliferative disorder (HV-LPD) is a rare EBV-induced photosensitive disease of childhood. Classic HV-LPD is self-limiting but the systemic variant is a progressive disease with extracutaneous symptoms and risk of developing into systemic lymphoma. Environmental factors and therapeutic approaches may influence skin histopathology, disease manifestation and clinical course as exemplified here. We report the case of a 10-year-old Tibetan boy with typical skin manifestation of classic HV-LPD but with an uncommon EBER-positive Th2 phenotype. Further, the patient showed both positive low-titer EBV- and SarsCov2-serologies without extracutaneous symptoms but acute and healed skin lesions triggered by UV light. EBV can be activated by SarsCov2 virus infections, which in this case might be a rare co-infectious systemic trigger for flare-ups. In the skin, clinical-pathological analysis suggests that HV-LPD begins as an unspecific erythema mediated by perivascular EBV-infected T-cells and dilated skin vessels followed by infection of the epidermis leading to EBV-untypical keratolytic papulovesicular degeneration. Further, UV-irradiation enhances the cytolytic effect in the skin, which clinically precipitates a prominent cutaneous photosensitivity. So far, systemic hydroxychloroquine sulfate induced a clinical remission under photoprotection compared to topical immunosuppression with mometasonfuroat and calcineurin inhibitors. Under antimalarial therapy, EBV-typical epidermal changes were not detectable and the perivascular EBER-positive T-cell infiltrate was reduced but not in complete remission. The proposed direct antiviral mechanisms of hydroxychloroquine in an acute EBV infectious setting might explain the beneficial, steroid-sparing therapeutic effect in HV-LPD but it may not protect the patients from flare-ups possible by underlying latent infection.