Paget’s disease in Senegal : two cases of a rare disease in black african populations

Mr. MD is a 72-years-old man, admitted for spontaneous, permanent, crushing type pain on the pelvis above the right hip evolving for two years, without night or morning stiffness but increasing with hearing loss, temporal and parietal headache. Physical examination showed a painful hip in active and passive mobilization. Pressure on iliac spines and lower lumbar and sacrococcygeal bones was painful. The patient showed no inflammatory syndrome. Serum calcium was normal. We noted an isolated increase in alkaline phosphatase levels to 401 IU/l. Radiographs showed bilateral heterogeneous sclerosis of the iliac bone with thickening lines and and almost disappearance of the right hip joint space. There was a marked thickening of the cortex on the femoral proximal third and thickening of the cranial vault. MRI showed cortical thickening of the right pelvic bone, a T1 hyper signal, and an intermediate T2 signal with fat/sat. This was pathognomonic of Paget's disease. The second patient is a 72-year-old man with no history, having intense pain on the right side of the lower limb. Physical examination showed no musculoskeletal deformity, but pain on palpation and mobilization of the right hip. Serum alkaline phosphatase (ALP) was raised to 4 times the normal range. Radiography showed cortical thickening of the ischial pubic branch, a heterogeneous sclerosis gypsy moth of the iliac wing, a steady narrowing of the femoral hip-spaced lines and thickening of the iliac ischial pubic. This aspect is pathognomonic of Paget's disease, the patient underwent treatment with zoledronic acid intravenously at 5 mg. The outcome was favorable up to 10 months with reduced pain (VAS = 2/10) and normal PAL.