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.