The Maritime Silk Road: case report of Neuro-Behçet’s disease from Somalia with positive HLA-B51 haplotype

Abstract

Behçet’s Disease (BD) is a multisystemic inflammatory disorder that commonly presents with oral and genital ulcers and uveitis, and can involve the nervous system i.e. Neuro-Behçet’s Disease (NBD). We present the first reported case of Neuro Behçet’s Disease (NBD) in a patient of Somali origin. A 34-year-old female from Somalia who had initially presented with headaches and Generalized Tonic-Clonic Seizures (GTCS) but was lost to follow-up. She represented with additional headache, neuropathic leg pain and ulcerated leg swellings with genital itching. Physical examination revealed hyperpigmentation and erythema nodosum on the lower limbs. Laboratory investigations revealed elevated ESR, positive HLA-B51 and a positive skin pathergy test. MRI brain scan revealed non-enhancing white matter hyperintensities in the right meso-diencephalic junction classical for NBD. She was commenced on immunosuppressive therapy with good response at one month. In conclusion, NBD is rare in sub-Saharan Africa. Our case report highlights that the disease is prevalent also on the Maritime Silk Road which includes Somalia.

Key words: Behçet’s disease, Neuro- Behçet’s disease, Sub-Saharan Africa, Somalia