Penile ischemic lesions associated with anti-phospholipid antibody syndrome

A 70-year-old man presented with fever, rigors, altered mental status and inguinal rash. Past medical history consisted of diabetes mellitus, hypertension, end-stage renal disease and prior ischemic stroke associated with anti-phospholipid antibody syndrome (APLAS) 1 year prior. Since his stroke, he was known to have cognitive impairment. He had been treated with warfarin, which he had stopped 2 months prior to admission following a fall. Physical examination revealed blood pressure of 155/76 mmHg, left shoulder petechiae and a painful, purple rash on the penis (Panel A). Magnetic resonance imaging of the brain was consistent with his age and a previous stroke. Computed tomography of the chest revealed bilateral basilar consolidations. Titers of IgG anticardiolipin were 83.2 g/l (previously > 150 g/l). Lupus anticoagulant and β2-glycoprotein were negative. Biopsy of the penile lesions revealed thrombotic vasculopathy manifesting with occlusion of blood vessels by necrotic material (Panel B; arrows). This was attributed to APLAS. Treatment with IV antibiotics, parenteral anticoagulation and corticosteroids was initiated. No new lesions developed and his condition improved over the course of several weeks.

Panel A Panel B

APLAS is diagnosed by a combination of characteristic clinical features and elevated antiphospholipid antibodies (APLA) present on at least two occasions, at least 12 weeks apart.^1,2 Antibodies can be of one or more of the following types: lupus anticoagulants, anticardiolipin IgG and/or IgM in medium or high titers or anti-β2 glycoprotein-I IgG and/or IgM in levels > 99th percentile. ² This syndrome causes venous, arterial, and small-vessel thrombosis and pregnancy morbidity. It may also manifest as cardiac valvular disease, renal thrombotic microangiopathy, hemolytic anemia, thrombocytopenia, and cognitive impairment. ³ Cutaneous manifestations of APLAS include livedo reticularis, skin ulcerations and rarely widespread cutaneous necrosis. ⁴ While penile ischemia with full recovery has been described in patients with APLAS, ⁵ this is a rare complication. It should be identified promptly as it can offer a diagnostic clue to the syndrome and direct treatment.

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