Periosteal new bone formation in polyarteritis nodosa. A syndrome involving the lower extremities

Abstract

Three patients with polyarteritis nodosa associated with periosteal new bone formation in the lower extremities are described. Seven cases with many similar features have been collected from the literature. Pain, swelling, and skin changes in the lower extremity with periosteal reaction seem to characterize one of the syndromes found in polyarteritis nodosa.Diffuse periosteal new bone formation is an uncommon feature of polyarteritis nodosa, but it does occur in association with a specific set of symptoms and findings.The unusual roentgenographic findings in case 1 prompted us to review the roentgenograms of other patients with polyarteritis nodosa. From 1968 to 1972, one hundred fifty-eight patients with a diagnosis of polyarteritis nodosa have been seen at the Mayo Clinic. This diagnosis was proposed on the basis of clinical findings and confirmed by histologic examination of biopsy specimens. Approximately half of these patients had had roentgenograms made of one or more of their extremities. A review of these roentgenograms disclosed two additional cases associated with periosteal new bone formation. REPORT OF CASESCase 1. A 46-year-old male teacher came to the Mayo Clinic in November 1971 because of pain and ulceration of the skin on the dorsum of the right foot. He had had recurrent ulcers on the right, and occasionally the left, ankle and foot since 1963, with exacerbations usually in the fall of each year. Investigation at another institution in March 1967 revealed electromyographic evidence of a peripheral neuropathy involving both lower extremities. Skin biopsy a t that time was reported to be negative. In December 1970 he was again extensively investigated at the same institution. T h e skin biopsy at that time revealed acute and subacute inflammation in the superficial dermis. T h e eosinophil count and the serum glutamic-oxalacetic transaminase (SGOT) value were increased.When the patient was first examined at the Mayo Clinic he complained of aching pain of such severity that he was unable to perform his teaching duties. There was mild swelling of the lower part of the right leg and the ankle, with a blotchy bluish discoloration of the skin. There were numerous circular brown macules and a large ulcer. L'aricose veins were not prominent and arterial pulsations were normal.Relevant laboratory data were: hemoglobin, 10.4 g/ 100 ml; erythrocyte count, 4,43O,OOO/cu mm; leukocyte count, 8,20O/cu mm with 11.5% eosinophils; peripheral blood smear, consistent with blood-loss anemia; erythrocyte sedimentation rate, 73 mm in 1 hour (Westergren); SGOT, 15 IU/liter; sulfobromophthalein (BSP) retention, 23% in 1 hour; serum protein electrophoresis, albumin 3.43 g/ 100m1, al-globulin 0.22 g/100 ml, =,-globulin 0.73

Lesions of juxtacortical origin (surface lesions of bone)

“…Stimulation of the periosteum by an inflammatory process results in a periosteal reaction. Periostitis has been described in patients with polyarteritis nodosa [12,21] and systemic lupus erythematosus [5].…”

Case number 34: Relapse of polyarteritis nodosa presenting as isolated and localised lower limb periostitis

“…Oral prednisone treatment was restarted with good clinical response. Cases of periostitis in patients with PAN (often localised PAN) have been reported, [1][2][3][4][5][6][7] although mostly in older publications. The periosteal reaction is most often found in the lower limbs, [1][2][3][4][5][6] and may or may not be accompanied by cutaneous lesions.…”

Polyarteritis nodosa presenting with a leg mass

“…There are few reports of PAN with periosteal new bone formation, although this is a described complication 5. In childhood, bone involvement in PAN is extremely rare; only two reported cases being found 6, 7.…”