Sweetâ??s syndrome is an uncommon skin disease characterized by painful polymorphic\nlesions associated with fever and neutrophilia. When biopsied, these lesions reveal a diffuse infiltrate\nof mature neutrophils in the papillary dermis. Several drugs can induce Sweetâ??s syndrome (so-called\ndrug-induced Sweetâ??s syndrome (DISS)) but reports of DISS associated with hydroxychloroquine\n(HCQ) are exceptionally limited. A 72-year-old Caucasian female patient with elderly-onset primary\nSjogrenâ??s syndrome (EOpSS) but low disease activity presented with an abrupt onset of painful\nnodular and papular erythematous skin lesions after two weeks of therapy with HCQ 400 mg. A\nhistological examination revealed a diffuse infiltrate of mature neutrophils in the papillary dermis,\nwithout vasculitis. After therapy with 25 mg/day prednisone and HCQ withdrawal, the cutaneous\nmanifestations disappeared. When prednisone was permanently discontinued, the primary Sjogrenâ??s\nsyndrome (pSS) manifestations worsened and therapy with HCQ 200 mg was reintroduced. In a\nfew days, the same skin lesions reappeared. Withdrawal of HCQ and a new cycle of prednisone\nresulted in their permanent disappearance. We reported a case of DISS following therapy with HCQ\nin a female patient affected by EOpSS. According to a literature review, this is the first report of\nthis association.
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