Systemic lupus erythematosus presenting as acquired factor XIII deficiency and spontaneous subdural haemorrhage

Factor XIII deficiency is a rare, potentially life-threatening bleeding disorder. While congenital disease is inherited in an autosomal recessive manner, acquired factor deficiency can be associated with autoimmune disease or malignancy. Clinical spectrum may range from mucocutaneous bleeding to intracerebral bleeding.

This case describes a 21-year-old woman who initially presented with low grade fever and cervical lymphadenopathy. In two weeks’ time she developed bilateral spontaneous subdural haemorrhages along with bilateral small and large joint arthritis. Investigations revealed an acquired factor XIII deficiency, positive antinuclear antibodies (ANA) titer and Coomb’s positive autoimmune hemolytic anaemia and was subsequently diagnosed to have systemic lupus erythematosus.

Intracerebral bleeding due to acquired factor XIII deficiency is a rare initial presentation of systemic lupus erythematosus in literature.