title: Reactive angioendotheliomatosis as a presenting cutaneous manifestation of Hughes syndrome.
abstract: BACKGROUND
  Antiphospholipid antibody syndrome is an autoimmune disorder associated with pregnancy complications, venous and/or arterial thrombosis and the presence of antiphospholipid antibodies. This syndrome is known to present with various cutaneous features, but association with reactive angioendotheliomatosis has been described rarely in the literature.
  
  
  RESULTS
  A woman in her thirties with a past history of three consecutive abortions developed purpuric, ulcerative plaque over the plantar aspect of the foot. Her biopsy showed marked expansion of dermal vasculature due to intravascular cellular proliferation suggestive of reactive angioendotheliomatosis. The intravascular cells stained positive for CD31. Her blood investigations showed positive lupus anticoagulant, antiphospholipid antibodies and anticardiolipin antibodies, leading to a diagnosis of antiphospholipid syndrome also known as Hughes syndrome.
  
  
  CONCLUSIONS
  We suggest that a hypercoagulable state caused the formation of intravascular thrombi leading to reactive angioendotheliomatosis. We report a case of Hughes syndrome with reactive angioendotheliomatosis as the first clinical cutaneous manifestation and treated satisfactorily with anticoagulants and immunomodulators.
authors.affiliation: Krishna Institute of Medical Sciences, Karad, India mangeshnikam@yahoo.com.
authors.forename: B
authors.initials: B
authors.lastname: Nikam
authors.affiliation: Krishna Institute of Medical Sciences, Karad, India.
authors.forename: S
authors.initials: S
authors.lastname: Shah
authors.affiliation: Krishna Institute of Medical Sciences, Karad, India.
authors.forename: M
authors.initials: M
authors.lastname: Kale
authors.affiliation: Krishna Institute of Medical Sciences, Karad, India.
authors.forename: V
authors.initials: V
authors.lastname: Jamale
mesh_terms: D000026:Abortion, Habitual / Q000097:blood / Q000473:pathology; D000328:Adult; D017153:Antibodies, Anticardiolipin / Q000097:blood; D017152:Antibodies, Antiphospholipid / Q000097:blood; D016736:Antiphospholipid Syndrome / Q000097:blood / Q000175:diagnosis* / Q000473:pathology; D004211:Disseminated Intravascular Coagulation / Q000097:blood / Q000473:pathology; D005260:Female; D006390:Hemangioendothelioma / Q000097:blood / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D006801:Humans; D016682:Lupus Coagulation Inhibitor / Q000097:blood; D010431:Pentoxifylline / Q000008:administration & dosage / Q000627:therapeutic use; D010975:Platelet Aggregation Inhibitors / Q000008:administration & dosage / Q000627:therapeutic use; D011247:Pregnancy; D011248:Pregnancy Complications / Q000097:blood / Q000188:drug therapy; D012878:Skin Neoplasms / Q000097:blood / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D019851:Thrombophilia / Q000097:blood / Q000473:pathology
chemical_list: D017153:Antibodies, Anticardiolipin; D017152:Antibodies, Antiphospholipid; D016682:Lupus Coagulation Inhibitor; D010975:Platelet Aggregation Inhibitors; D010431:Pentoxifylline
keywords: Antiphospholipid syndrome; reactive angioendotheliomatosis