Pemphigus in Mali: a study of 30 cases.

Pemphigus has been largely studied in developed countries (North America and Europe) and in Brazil. In these geographical settings, pemphigus presents two very different epidemiological and clinical patterns. Little is known about pemphigus in other regions of the world, particularly in Africa. We report here a study of 30 cases of pemphigus observed in Bamako, Mali. Our data suggest that pemphigus in this area presents a distinctive pattern. Our cases of pemphigus were diagnosed on the basis of clinical, histological and direct immunofluorescence studies. We estimated the annual incidence in the Bamako region to be 0.29 cases per 100,000 inhabitants. There was no endemic focus in Mali. The disease was observed mainly in women (24 of 30; 80%), especially those older than 40 years (mean age, 46.7 years), and in the Fulani ethnic group (10 of 30; 33%). Our study group was composed of 25 cases of pemphigus foliaceus (PF) (83%), four cases of pemphigus vulgaris and one case of pemphigus vegetans. Pustules with hypopyon were observed in 11 patients (37%). A diffuse verrucous change in the skin was noted in four cases of erythrodermic PF. In 16 patients with PF, localized verrucous lesions mimicking seborrhoeic keratoses were observed when oral corticosteroid treatment was decreased. Histopathological examination demonstrated eosinophilic spongiosis in 50% of patients. These data suggest that pemphigus in Mali differs from the two main known patterns of the disease: the North American/European one, and the Brazilian pattern, with which it shares the predominance of superficial forms but otherwise differs in many features.