Neonatal lupus erythematosus presenting as Stevens-Johnson syndrome

Abstract Neonatal lupus erythematosus (NLE) is a rare acquired condition caused by the transplacental passage of maternal autoantibodies. It is characterized by cutaneous, cardiac, hepatobiliary, hematological and neurological involvement. Cutaneous findings of NLE are variable, but few reports in the literature describe the presence of erosions or epidermal loss in NLE. Herein, we describe the case of an infant with NLE presenting as Stevens-Johnson syndrome (SJS), with cardiac, hematologic, hepatobiliary and neurologic abnormalities. The characteristic features by which to differentiate SJS-like NLE from infant SJS include subacute course, cardiac abnormalities, positive serology tests and direct immunofluorescence, histopathologic findings with melanin incontinence, periadnexal infiltrates, and mucin deposit. Though rare, it is important to consider lupus erythematosus as a potential cause of acute syndrome with focal epidermal necrosis or pan-epidermolysis.