Nasal Skin Necrosis: A Very Rare Manifestation of Antiphospholipid Syndrome
  • Carolina Oliveira Ourique
    Internal Medicine Department, Centro Hospitalar de São João, Oporto
  • Inês Chora
    Internal Medicine Department, Centro Hospitalar de São João, Oporto
  • Susana Guimarães
    Anatomical Pathology Department, Centro Hospitalar de São João, Oporto
  • Sara Júlio
    Otorhinolaryngology Department, Centro Hospitalar de São João, Oporto
  • Paula Vaz-Marques
    Internal Medicine Department, Centro Hospitalar de São João, Oporto

Keywords

Cutaneous necrosis, antiphospholipid syndrome, cryoglobulinaemia

Abstract

A 23-year-old woman, a smoker and oral oestrogen user, presented with nasal necrosis. No other symptoms or local trauma were described. Relevant laboratory findings included complement consumption, positive lupus-anticoagulant assay, increased rheumatoid factor and positive cryoglobulins. Screening for autoimmune conditions, haematological malignancies and infectious diseases was negative. Histological examination of the nasal skin showed small vessel occlusion without vasculitis. Later, a second positive lupus-anticoagulant assay supported the diagnosis of antiphospholipid syndrome. The patient improved with glucocorticoids and anticoagulation. This case report describes an unusual manifestation of antiphospholipid syndrome in a patient with cryoglobulinaemia contributing to the thrombotic event and highlights the importance of recognizing these overlapping disorders.

VIEW THE ENTIRE ARTICLE

References

  • Miyakis S, Lockshin MD, Atsumi T, Branch DW, Brey RL, Cervera R, et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost 2006; 4:295-306.
  • Giannakopoulos B, Krilis S. The pathogenesis of Antiphospholipid Syndrome. N Engl J Med 2013; 368:1033-1044.
  • Ramos-Casals M, Stone J, Cid M, Bosch X. The cryoglobulinaemias. The Lancet 2012; 379:348-360.
  • Shachaf S, Yair M. The correlation between antiphospholipid syndrome and cryoglobulinemia: case series of 4 patients and review of the literature. Rev Bras Reumatol Engl Ed. 2016; 56(1):2-7.
  • Cervera R, Khamashta MA, Shoenfeld Y, Camps MT, Jacobsen S, Kiss E, et al. Morbidity and mortality in the antiphospholipid syndrome during a 5-year period: a multicentre prospective study of 1000 patients. Ann Rheum Dis. 2009; 68(9):1428-32.
  • García-Carrasco M, Ramos-Casals M, Cervera R, Trejo O, Yague J, Sisó A, et al. Cryoglobulinemia in systemic lupus erythematosus: prevalence and clinical characteristics in a series of 122 patients. Semin Arthritis Rheum. 2001; 30(5): 366-73.
  • Views: 1310
    HTML downloads: 4335
    PDF downloads: 376
    Figure 1. downloads: 0
    Figure 2. downloads: 0
    Figure 3. downloads: 0
    Figure 4. downloads: 0
    Clinical image description downloads: 0


    Published: 2017-03-27
    Issue: Vol. 4 No. 2 (view)


    How to cite:
    1.
    Ourique CO, Chora I, Guimarães S, Júlio S, Vaz-Marques P. Nasal Skin Necrosis: A Very Rare Manifestation of Antiphospholipid Syndrome. EJCRIM 2017;4 doi:10.12890/2016_000534.