Cutaneous polyarteritis nodosa in children.
The purpose of this study was to present the clinical courses and histologic findings of 4 children with cutaneous vasculitis characterized by tender cutaneous nodules and fever in the absence of major organ involvement. We conducted a retrospective chart review of 4 patients with cutaneous vasculitis followed up for a mean of 68 months (range, 12-114 months). The patients included 3 boys and 1 girl (ages at onset, 2-10 years). Clinical and laboratory manifestations included tender erythematous cutaneous nodules (n = 4/4), fever 39 degrees C or higher (4/4), nondeforming arthritis (3/4), leukocytosis and elevated erythrocyte sedimentation rate (4/4), positive antinuclear antibodies (1/4), and elevated streptococcal enzymes (3/4). Skin biopsy results showed inflammation of medium-sized cutaneous arteries with a mixed inflammatory cell infiltrate consistent with cutaneous polyarteritis nodosa (4/4). Patients were treated with prednisone with good initial response, but exacerbation occurred once prednisone was tapered. Additional medications given were methotrexate (2/4), dapsone (2/4), colchicine (1/4), and cyclophosphamide (1/4). One patient is in clinical remission after 48 months of disease; the others have continuing disease that requires treatment. Patients with evidence of streptococcal infection received oral penicillin prophylaxis; two of the three patients had recurrent attacks of vasculitis despite penicillin. No patients have developed major organ system involvement after 12 to 114 months of follow-up. Cutaneous polyarteritis nodosa in children is a recognizable entity characterized by painful nodules, fever, absence of major organ involvement, and chronic or recurrent course. Patients should be screened for streptococcal infection and treated with antibiotics when needed.
Fathalla
Basil M
BM
Division of Pediatric Rheumatology, Tufts University/New England Medical Center, Boston, Massachusetts, USA.
Miller
Laurie
L
Brady
Stephen
S
Schaller
Jane G
JG
D019072:Antibiotic Prophylaxis; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D010406:Penicillins / Q000627:therapeutic use; D010488:Polyarteritis Nodosa / Q000175:diagnosis* / Q000188:drug therapy / Q000517:prevention & control; D012189:Retrospective Studies; D055502:Secondary Prevention
D010406:Penicillins