Livedoid vasculopathy and hypercoagulability in a patient with primary Sjögren's syndrome

Abstract

Background A 31-year-old woman presented with a 5-year history of painful ulcerations, palpable purpura, porcelain-white atrophic scars of the malleolar region and dorsal aspect of the feet, livedo reticularis on the limbs, arthralgia, xerophthalmia, and xerostomia. Methods Skin biopsy revealed vessel wall hyalinization and thrombosis of the microvasculature with a very scarce dermal inflammatory infiltrate. Biopsy of the oral mucosa showed mononuclear infiltration of an intralobular duct of a salivary gland.Results Laboratory studies, including autoantibodies and inflammation markers, were normal, except for a positive rheumatoid factor. Coagulation screening revealed C677T methylenetetrahydrofolate reductase (MTHFR) mutation, with a normal serum homocysteine.The patient was treated with oral methylprednisolone (32 mg/day with progressive reduction) and enoxaparin (20 mg/day subcutaneously), with complete ulcer healing within 4 months. ConclusionLivedoid vasculitis or vasculopathy has not been referred to previously in association with Sjögren's syndrome, but may be associated with other autoimmune disorders and anomalies of coagulation, namely factor V Leiden mutation, protein C deficiency, and MTHFR mutation, associated or not with hyperhomocysteinemia, a condition that seems to confer an increased risk of recurrent arterial and venous thrombosis. We stress the importance of anticoagulant therapy for ulcer healing and for the prevention of other thrombotic events.

Vasculitis in Sjögren’s Syndrome

“…As has been previously described [20], palpable purpura with leukocytoclastic vasculitis is occasionally the presenting problem in Sjögren’s syndrome [21]. Nail fold capillaroscopic findings had nonspecific abnormalities in about one third of 61 Sjögren’s syndrome patients.…”

Update of management of connective tissue diseases: livedoid vasculopathy

“…The patient had an associated mutation of the enzyme methylenetetrahydrofolate reductase that results in hyperhomocysteinemia, which is related to a hypercoagulable state (20). The patient had an associated mutation of the enzyme methylenetetrahydrofolate reductase that results in hyperhomocysteinemia, which is related to a hypercoagulable state (20).…”

A Fulminant Case of Renal Vein Thrombosis in a Patient with Autoimmune Disorder and Membranous Nephropathy

“…There are a few affirmative writings on steroid therapy in both SjS-and GD-associated MN (8,9). Because the beneficial effects of steroids are not only for MN but also for thyrotoxicosis and hypercoagulability and venous inflammation with SjS (10), it should be considered that immunosuppressive therapy including steroids is attempted against the thrombogenic tendency in those patients.…”