Mixed connective tissue disease associated with skin defects of livedoid vasculitis

Clin Rheumatol. 2000;19(5):381-4. doi: 10.1007/s100670070032.

Abstract

A 21-year-old woman who had a 2-year history of mixed connective tissue disease (MCTD) developed rapidly evolving ulcers consistent with livedoid vasculitis (LV) in all distal extremities. She presented clinically with Raynaud's phenomenon, polyarthritis and swollen hands; serologically with high titres of ANA and anti-nRNP; and immunogenetically with HLA-DR4 and HLA-DR53. Although there was initial success in treatment except for the skin defects over the ankles, the patient died from disseminated intravascular coagulation. We suggest that LV may be a poor prognostic manifestation in MCTD.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Antibodies, Antinuclear / analysis
  • Biopsy
  • Diagnosis, Differential
  • Female
  • HLA-DR Antigens / immunology
  • HLA-DR4 Antigen / immunology
  • HLA-DRB4 Chains
  • Humans
  • Mixed Connective Tissue Disease / complications*
  • Mixed Connective Tissue Disease / immunology
  • Mixed Connective Tissue Disease / pathology
  • Recurrence
  • Ribonucleoproteins, Small Nuclear / immunology
  • Skin Diseases, Vascular / complications*
  • Skin Diseases, Vascular / etiology
  • Skin Diseases, Vascular / immunology
  • Skin Diseases, Vascular / pathology
  • Skin Ulcer / etiology*
  • Skin Ulcer / pathology

Substances

  • Antibodies, Antinuclear
  • HLA-DR Antigens
  • HLA-DR4 Antigen
  • HLA-DR53
  • HLA-DRB4 Chains
  • Ribonucleoproteins, Small Nuclear