Cutaneous polyarteritis nodosa in children

Fathalla, Basil M.; Miller, Laurie C.; Brady, Stephen; Schaller, Jane G.

doi:10.1016/j.jaad.2005.03.065

Cited by 59 publications

(50 citation statements)

References 18 publications

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“…Penicillin should be considered in children with increased ASO titres [5, 7, 9, 16, 56]. Recent case series report success with low-dose methotrexate, cyclophosphamide, intravenous immunoglobulin, and biologic therapies [3, 4, 11, 57]. Eleftheriou and colleagues report the largest cohort of pediatric patients treated with biologic therapy.…”

Section: Discussionmentioning

confidence: 99%

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

Bansal

Houghton

2010

Arthritis

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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.

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Section: Discussionmentioning

confidence: 99%

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

Bansal

Houghton

2010

Arthritis

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“…The most common inciting agent identified is Group A β hemolytic Streptococcus. A preceding upper respiratory infection with Streptococcus has been detected in many adults and children, employing anti‐streptolysin O antibody titers or throat swab cultures 8,9,15–17 . Antecedent streptococcal upper respiratory infections were noted in all four children with CPAN in one analysis 18 .…”

Section: Etiologymentioning

confidence: 99%

Cutaneous polyarteritis nodosa: a comprehensive review

2010

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Cutaneous polyarteritis nodosa is a rare form of vasculitis relating to small-to-medium-sized arteries. Its etiology is unknown. Clinical manifestations include tender subcutaneous nodules, livedo reticularis, cutaneous ulcers and necrosis. Although it is distinct from systemic polyarteritris nodosa in that it lacks significant internal organ involvement, extra-cutaneous manifestations may be evident. Commonly encountered symptoms include fever, malaise, myalgias, arthralgias, and paresthesias. Exclusion of systemic polyarteritis nodosa is essential in diagnosis. The clinical course is chronic with remissions, relapses, and a favorable prognosis. Mild cases may resolve with nonsteroidal anti-inflammatory drugs. If more severe, treatment with systemic corticosteroids generally achieves adequate response; however, adjunctive therapy is often necessary to allow reduction in steroid dosage.

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“…Remissions have been reported, but upon tapering the steroid dose exacerbations of CPAN are generally reported [3]. Steroid sparing therapies reported for CPAN include hydroxychloroquine, dapsone, azathioprine, methotrexate, IvIg, and cyclophosphamide [10][11][12][13]. Although immunosuppressives have been reported to be effective in CPAN, there are no controlled prospective trials to evaluate their efficacy.…”

Section: Discussionmentioning

confidence: 99%

Refractory cutaneous polyarteritis nodosa with threat of loss of feet: successful treatment with rituximab

Stephan¹,

Minna²

2017

J Rheumatol Orthop

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Cutaneous Polyarteritis Nodosa (CPAN) is a rare, single organ vasculitis of unknown etiology which presents with livedo reticularis and tender nodules involving the lower extremity in 97% of cases. Diagnosis is established by skin biopsy demonstrating segmental leukocytoclastic vasculitis and fibrinoid necrosis with workup excluding any systemic involvement ( Figure 1). The presence of IgM antiphosphatidylserine-prothrombin complexes has led to the hypothesis that CPAN is a localized arthrus reaction. CPAN is characterized as a benign, chronic, relapsing vasculitis to be treated conservatively. As a single organ vasculitis, CPAN can affect arteries of any size. Due to CPAN's predilection for the lower extremities, involvement of the limb at or below the ankle's major arteries may result in loss of digits and potentially feet. The case presented involves CPAN in which the main arteries to the feet are occluded. The patient had been refractory to therapy with findings of early digital necrosis. Following adequate skin biopsy to establish a diagnosis, we initiated targeted therapy with rituximab, azathioprine, and high dose steroids with reversal of the ischemic process. It is the authors' opinion that CPAN should be treated more aggressively from initial diagnosis to prevent morbidity and potentially abort a chronic relapsing course.

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Cutaneous polyarteritis nodosa in children

Cited by 59 publications

References 18 publications

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

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

Cutaneous polyarteritis nodosa: a comprehensive review

Refractory cutaneous polyarteritis nodosa with threat of loss of feet: successful treatment with rituximab

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