Diagnostic and treatment difficulties in systemic lupus erythematosus coexisting with sickle cell disease

Patients with Sickle cell disease present with a wide range of symptoms and signs which overlap with other chronic illnesses. This often leads to a delay in diagnoses of the associated disorder.

In view of the high prevalence of SCD in our environment, it is important to know that this association can occur and should be considered when patients present with pyrexia, arthritis, seizures and a recurrent rash.

We present an 8-year-old boy with sickle cell disease who presented with recurrent fever, back pains, and 'cutaneous eruptions' to multiple drugs. He had several admissions within two years due to above symptoms. Examination at the onset of the illness revealed a young boy with fever, no jaundice, and periorbital oedema and generalized lymphadenopathy. He had scanty fluffy hair and post inflammatory hyper pigmentation on the trunk and extremities. He also had a hepatomegaly. He was started on Ibuprofen and ceftraixone to which he reacted. He had corticosteroids and antimalarials and improved. During the 2-year-follow up period he was admitted for recurrent seizures, arthritis and a leg ulcer. On last visit to hospital, he developed a malar rash. Discoid rash with mouth ulcers. A diagnosis of SLE was made in the patient.

There are only 23 reported cases of SLE occurring in patients with sickle cell disease in literature, suggesting that the association is rare, it should be considered in patients with sickle cell disease presenting with pyrexia, rash and seizures.