Jeffrey P. Callen, MD
Early recognition and prevention of extended morbidity is facilitated by dermatologic examination.
Polyarteritis nodosa (PAN), a rare, multisystem inflammatory condition occurring primarily in middle-aged adults, appears even more rarely in children (< 5% of total cases). Aggressive intervention with systemic corticosteroids and immunosuppressive agents appears to alter the disease course.
Two studies of childhood PAN have recently been published. The first is a case report of a 33-month-old who presented with fever, a necrotic skin lesion, an oral ulceration, and cervical lymphadenopathy. Initial biopsy samples were nonspecific, but following bowel perforation and development of a new ulceration, further biopsies revealed necrotizing vasculitis involving small- and medium-sized vessels in the mid-dermis and subcutaneous fat. Treatment included infliximab and corticosteroids plus pulsed monthly cyclosphosphamide and methylprednisolone for 7 months, followed by 12 months of oral mycophenolate mofetil and, finally, 3 months of subcutaneous methotrexate. Remission was achieved without relapse in 3 months after therapy ended.
The second report is a retrospective analysis in 69 patients treated over 32 years at a tertiary care center. Clinical involvement at presentation was cutaneous (88%), febrile (86%), myalgic (82%), renal (19%), severe gastrointestinal (10%), and neurological (10%). Cutaneous manifestations included livedo reticularis in 50%, purpura (40%), skin nodules (27%), and ulcerations (3 superficial, 3 deep), as well as edema, Raynaud phenomena, and “other vasculitis.” Delays in diagnosis grew shorter over the study period. The most frequent misdiagnosis was infectious disease. Therapy included systemic corticosteroids in all patients, cyclosphosphamide in most, and azathioprine, methotrexate, mycophenolate mofetil, infliximab, or rituximab in some. The relapse rate was 34% and the mortality rate 4%. Relapse was related to gastrointestinal involvement, delay in diagnosis, and higher cumulative doses of cyclosphosphamide. Long-term effects of therapy (e.g., sterility or subsequent malignancy) were not reported; the authors suggest that earlier intervention lowered mortality in these patients relative to earlier studies.
Citation(s):
Beckum KM et al. Polyarteritis nodosa in childhood: Recognition of early dermatologic signs may prevent morbidity. Pediatr Dermatol 2013 Aug 13.
Eleftheriou D et al. Systemic polyarteritis nodosa in the young: A single-centre experience over 32 years. Arthritis Rheum 2013 Jun 10.
