Cutaneous Polyarteritis Nodosa in Childhood: A Case Report and Review of the Literature

Abstract

Polyarteritis nodosa is a rare vasculitis of childhood. Cutaneous PAN (cPAN) is limited to the skin, muscles, joints, and peripheral nerves. We describe a 7.5-year-old girl with cPAN presenting initially as massive cervical edema who later went on to develop subcutaneous nodules, livedo reticularis, myositis, arthritis, and mononeuritis multiplex. The use of corticosteroids resulted in initial clinical improvement, but symptom recurrence necessitated disease modifying antirheumatic drugs and biologic therapy. We review a further 119 reports of biopsy proven cPAN in the literature. A majority of patients (96.6%) had cutaneous involvement; musculoskeletal involvement was common and included both articular (58.0%) and muscular (42.9%) symptoms, and nervous system involvement was least common (18.5%). Corticosteroids were used in the majority of patients (85.7%), followed by use of disease modifying antirheumatic drugs (33.0%), nonsteroidal anti-inflammatory drugs (10.7%), and intravenous immunoglobulin (9.8%). Therapy of cPAN with biologics has only been reported in 2 patients, and we report the first patient treated with Rituximab. A diagnosis of cPAN should be considered in a child with fever, vasculitic rash, and musculoskeletal symptoms. Most children respond to corticosteroids and have a benign course, but some require disease modifying antirheumatic drugs and biologic therapies.

Long-term outcome of children with pediatric-onset cutaneous and visceral polyarteritis nodosa

“…In particular, when considering a looser definition of "cutaneous PAN", as also proposed in many other publications [11,13,16,26], we found a few cases where children with "cutaneous PAN" and no visceral involvement went on to develop severe complications, such as digital necrosis, ending up with extended periods of glucocorticoid therapy and immunosuppressive drugs and having long-lasting disease activity. We conclude that the organ distribution-based distinction between cutaneous and visceral childhood PAN had little prognostic power in this series.…”

“…In adults, cutaneous PAN likely appears as an indolent disease, and some authors limit the definition of cutaneous PAN to cutaneous involvement without fever, myalgia, arthralgia or peripheral neuropathy (except if present at the same localization as skin lesions) [12]. In contrast, a review of 119 children with cutaneous PAN found that most children had fever and elevated acute-phase reactants, and that musculoskeletal features, joint involvement and peripheral neuritis were frequent (43%, 58% and 18%, respectively) [13].…”

“…Patients requiring glucocorticoids had higher leukocyte counts at diagnosis (19.5 ± 8 × 10 9 /L −1 [range: 7-38] vs 13.2 ± 6 × 10 9 /L −1 [range: [6][7][8][9][10][11][12][13][14][15][16][17][18][19][20], respectively, P = 0.06) and higher neutrophil counts (15.6 ± 8 × 10 9 /L −1 vs 9.3 ± 5 × 10 9 /L −1 , respectively, P = 0.06) than other patients, and fulfilled Ankara criteria in 14 out of 22 cases versus 2 out of 7 patients who remained glucocorticoid-free (odds ratio 4.38 at 95% CI [range: 0.53-53], P = 0.1). There were no differences on other variables.…”

A case report of cutaneous polyarteritis nodosa in siblings

“…However, CPAN is uncommon in children; there are only approximately 140 cases in the literature [3]; and sibling cases have never been reported. Therefore, our siblings' cases might suggest the possibility that E148Q MEFV mutation and/or HLA-A24, -DR15 alleles could influence the development of CPAN, although these commonly observed HLA alleles might not be the major factor for the onset in siblings.…”

“…kidney, heart, and liver [1][2][3]. Clinical manifestations of CPAN include tender subcutaneous nodules, livedo reticularis, cutaneous ulcers, and necrosis [1,2].…”