Introduction: The antiphospholipid syndrome (APS) is an autoimmune multisystem disease characterized by vascular thromboembolic events and pregnancy morbidity in the presence of elevated titers of antiphospholipid antibodies (aPL). The prevalence of dermatologic manifestations in APS reaches up to 55% of the patients, depending on the series. Extensive cutaneous necrosis is a very rare skin manifestation, usually associated with catastrophic APS (CAPS), and never reported in children.
Case description: A 16 year-old girl with a medical history of systemic lupus erythematosus (SLE) and positive aPL was admitted to our department because of E.Coli ESBL pyelonephritis. On her 5th day of hospitalization she developed an acute, rapidly progressive extensive cutaneous necrosis in both her thighs, necessitating a very complex multidisciplinary approach and eventually skin grafting. This catastrophic presentation was not associated with CAPS or other superimposed thromboembolic states.
Discussion: The reported prevalence of cutaneous necrosis in a few adult registries is about 2%. It is usually associated with CAPS which is characterized by multiorgan failure related to wide spread thrombotic microangiopathy. This is the first reported case in the literature of an extensive cutaneous necrosis in children with SLE and aPL. We present this case in order to raise the awareness of this catastrophic, life-threatening clinical entity and provide tools for rapid diagnosis and treatment.
