Clinical presentations of Behçet's disease among Nigerians: a 4-year prospective study.

BACKGROUND

Behçet's disease (BD) is a chronic, multisystem, inflammatory pan-vasculitis of unknown etiology, with heterogeneous presentations in different parts of the world. It commonly involves the mucosae, skin, joints, eyes, and central nervous system. Behçet's disease is considered to represent an autoimmune reaction triggered by a yet to be identified infectious agent in a genetically predisposed person. It most commonly affects persons of Mediterranean or Far Eastern origin and is considered rare among Black Africans.

OBJECTIVES

This study was conducted to document clinical presentations of BD in Nigerians.

METHODS

A prospective study of the clinical and laboratory parameters of patients who attended dermatology and rheumatology clinics between 2007 and 2011 was carried out.

RESULTS

Fifteen patients (nine males, six females) were diagnosed with BD at a mean age of 33 years. The mean age of onset of disease was 27 years. Oral ulceration was present in 100% of subjects and was the most frequent onset lesion, followed by genital ulceration in 93%. Skin, joint, vascular, gastrointestinal, and central nervous system (CNS) lesions occurred in 87, 80, 33, 20, and 13% of patients, respectively. Pathergy tests were positive in 38% of patients. Psychomorbidity was present in 60%. Remission was achieved with corticosteroids, nonsteroidal anti-inflammatory drugs, and disease-modifying anti-rheumatic drugs. Mortality was zero, but 27% of patients were disabled by blindness and 7% by CNS involvement.

CONCLUSIONS

This is the first report on BD in a sample of Nigerians. The clinical phenotype of BD follows the Middle Eastern pattern. The small number of patients may reflect the rarity of BD in Nigerians or the poor ascertainment of BD by relevant clinicians.