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.
Learning points:
Nasal skin necrosis is an uncommon event with a wide spectrum of aetiologies.The rare association of antiphospholipid syndrome with cryoglobulinaemia may act synergically in small-medium vessel occlusion, with skin involvement being the most common manifestation.Anticoagulation and immunosuppression of antiphospholipid syndrome with cryoglobulinaemia is mandatory.