Hydroxichloroquine-induced steven johnson syndrome. A case report

ABSTRACT

Background: Systemic lupus erythematosus (SLE) is an autoimmune disease with a multiple organs-involvement. SLE pathogenesis is sustained by innate and adaptive immune system disregulation, complement activation, immune complexes, and tissue inflammation. The pattern of clinical manifestations is heterogenous and changes over time. Hydroxychloroquine (HCQ)is a primary drug in the treatment of SLE. It modulates the immune response by inhibiting B cell receptor and TLR signaling and activation. Clinical Case This is a case report of a 61-years-old female affected by SLE with recurrent oral aftosis, Lupus anti-coagulant antibodies and previous pleuritis in remission. The patient had a long course treatment with low-dose oral corticosteroids (5 mg prednisone) and HCQ for three weeks. After 20 days of treatment with HCQ she developed an abrupt cutaneous manifestation consisting in painful and infiltrating papules which appeared over the upper trunk and limbs, redness, scales lesions on palms and soles, lip swelling, painful conjunctivitis and mild disphagia. Slowly, progressive improvement of cutaneous and mucosal signs was observed after drug discontinuation, two days after appearance of clinical manifestations, and introducing medium-high dose of oral corticosteroids therapy. Results: Clinical exams, performed in the acute phase highlighted a marked lymphocyto penia and raise of Reactive C Protein (RCP). Nicolsky sign was slightly positive. After discontinuation of HCQ the patient did not show cutaneous or infective sequelae due to prompt identification of Steven-Johnson Syndrome (SJS). The patient replaced HCQ with the increase of the mainteinance dose of oral corticosteroids. Skin tests were not perfomed for the risk of SJS exacerbation and concomitant corticosteroids therapy. Conclusions: Like all medications, HCQ has side effects which may occur in autoimmune disease patients. The main undesirable effects observed are digestive disorders and skin manifestations. Although skin diseases are common side effects of HCQ severe drug hypersensitivity reaction is a rare condition described in few case reports of DRESS and SJS. Despite the rare occurrence of these reactions, we observed other cases of drug reactions during the pandemic breakthrough in Italy when HCQ was widely used for the treatment of COVID-19.