A 28-year-old woman, 34 weeks pregnant, with previously diagnosed antiphospholipid syndrome, presents with vesicular tongue lesions treated as herpes outbreak and new onset of preeclampsia. Tongue biopsy preformed postpartum after induction of labor for preeclampsia reveals tongue infarction.
Case Report
A 28-year-old female, gravida 3 P 1, 34 weeks pregnant, presented to the labor and delivery suite with a 10 day history of lingual vesicular lesions and mouth soreness. She had a history of herpes simplex virus infection, with relatively frequent outbreaks, usually on her lower lip but never on her tongue. Her primary care physician had started her on valacyclovir orally for a presumed herpes outbreak. However, the lingual lesion became very painful and she was having difficulty tolerating oral fluid or food intake.
Her past medical history was significant for a second-trimester fetal demise 4 years previously. She was subsequently found to have persistently positive lupus anticoagulant and high-titer antiphospholipid antibodies (aPLs), and the diagnosis of antiphospholipid syndrome (APS) was made. She became pregnant again while on aspirin 81 mg and prophylactic enoxaparin 40 mg subcutaneously (SC) daily. She had an uneventful second pregnancy and delivered at 37 weeks of gestation. She had postpartum thromboprophylaxis with enoxaparin 40 mg SC daily for 6 weeks. During the current, third pregnancy, she was managed with a prophylactic dose of enoxaparin 40 mg SC and aspirin 81 mg daily. The enoxaparin dose was increased to 60 mg SC daily because her antifactor Xa activity was found to be decreased at 32 weeks of gestation.
On examination, purple discoloration and blisters were noted on the right lateral aspect of the tongue and on the superior and inferior surfaces ( Figure , A). Her blood pressure was elevated to 140/92 mmHg, and urinalysis demonstrated 30 mg of protein. Platelets were decreased to 111,000/μL. Concern for preeclampsia prompted hospital admission for 24-hour urine protein collection and observation. The treatment with valacyclovir for the lingual lesion was continued. The following day, her 24-hour urine protein collection demonstrated significant proteinuria (360 mg per 24 hours), and her platelet count dropped to 80,000/μL. Her blood pressure remained elevated, and she was induced with oxytocin for presumed atypical hemolysis-elevated liver enzymes-low platelets syndrome (HELLP). She had an uncomplicated labor and delivered vaginally a healthy male baby with Apgar scores of 9 and 9 for 1 and 5 minutes, respectively, and weighing 2890 g. Placental pathology demonstrated decidual vasculopathy and 3-vessel cord with focal myonecrosis.
Persistence of the painful lingual lesion despite treatment prompted a lingual biopsy, which revealed recent thrombi in small- to medium-sized vessels, leading to ischemic necrosis of the adjacent striated muscle and adjacent acute inflammation. Mucosal necrosis and ulceration were also noted ( Figure , B); these findings were consistent with lingual infarction.
The patient was restarted on therapeutic enoxaparin at 60 mg every 12 hours. On a follow-up visit 2 weeks postpartum, her lingual lesion was markedly improved, and the lesion completely resolved 4 weeks after delivery ( Figure , C).
APS is a clinicopathological diagnosis characterized by the presence of vascular thrombosis and/or pregnancy morbidity along with positive antibodies directed against phospholipids or phospholipid-binding proteins (aPLs), present on confirmatory testing after 12 weeks. Arterial infarction and the subsequent gangrene of the tongue has previously been described in patients with systemic lupus erythematosus, atrial fibrillation, giant cell arteritis, or radiation but never in patients with APS. In addition, liver, bone, and gut infarction in association with APS and HELLP syndrome have been reported. Thrombotic microangiopathy is a pathological feature of catastrophic antiphospolipid syndrome (when 3 or more organ systems are affected by thromboses in less than a week), however, with high frequency of kidney, skin, pulmonary, and central nervous system involvement, whereas tongue pathology has never been reported before.
Other unusual sites of thrombosis have been reported in pregnant women with APS, including cerebral venous sinus thrombosis in the first trimester of pregnancy and ovarian vein thrombosis in the postpartum period. Hepatic infarction, a rare condition caused by the dual blood supply to the liver, acute noninflammatory pancreatitis, nonbacterial thrombotic endocarditis, Snedon’s syndrome (noninflammatory occlusive arteriopathy of small- and medium-sized arteries of the skin and the brain leading to cerebrovascular disease and livedo reticularis), avascular bone necrosis, and adrenal vein thrombosis resulting in Addison’s disease have also been reported.
We believe that any unusual lesion in a pregnant patient with APS, a condition with high morbidity and mortality, should be investigated thoroughly to establish an accurate diagnosis and initiate prompt therapeutic intervention.
Acknowledgments
Contributions to this work were made by the following authors: Dr Lekovic was involved in the literature search, data collection, and writing the report; Dr Friedman performed the pathology analysis and provided pathology photographs; and Dr DeSancho was involved in the literature search, editing the report, and supervision.
The authors report no conflict of interest.
Stay updated, free articles. Join our Telegram channel
Full access? Get Clinical Tree
