title: 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.
  
  
  CONCLUSIONS
  Livedoid 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.
authors: Clinics of Dermatology, Hematology, and Rheumatology, University Hospital, Coimbra, Portugal. raquel.tavares.cardoso@iol.pt
authors: Raquel
authors: R
authors: Cardoso
authors: Margarida
authors: M
authors: Gonçalo
authors: Oscar
authors: O
authors: Tellechea
authors: Rosa
authors: R
authors: Maia
authors: Catarina
authors: C
authors: Borges
authors: J A Pereira
authors: JA
authors: Silva
authors: Américo
authors: A
authors: Figueiredo
mesh_terms: D000328:Adult; D000925:Anticoagulants / Q000008:administration & dosage; D003937:Diagnosis, Differential; D017984:Enoxaparin / Q000008:administration & dosage; D005260:Female; D006801:Humans; D007279:Injections, Subcutaneous; D007871:Leg Ulcer / Q000097:blood / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D012859:Sjogren's Syndrome / Q000097:blood / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D013577:Syndrome; D019851:Thrombophilia / Q000097:blood / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D014657:Vasculitis / Q000097:blood / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology
chemical_list: D000925:Anticoagulants; D017984:Enoxaparin