Successful Treatment in Two Cases of Steroid-Dependent Cutaneous Polyarteritis nodosa with Low-Dose Methotrexate

Schartz, Noël Emile Célestin; Alaoui, Siham; Vignon-Pennamen, Marie Dominique; Cordoliani, F.; Fermand, Jean Paul; Morel, P; Rybojad, M.

doi:10.1159/000051785

Cited by 38 publications

(19 citation statements)

References 5 publications

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“…When there is a lack of response to standard treatment or concerns about possible steroid toxicity, intravenous immunoglobulin (IV-Ig) has been successfully used [110]. Some success has also been reported with methotrexate [111], colchicine and dapsone [112], cyclophosphamide [98], pentoxifylline [113] and chloroquine [114]. …”

Section: Cutaneous Polyarteritis Nodosamentioning

confidence: 99%

Medium-size-vessel vasculitis

Dillon¹,

Eleftheriou²,

Brogan³

2009

Pediatr Nephrol

110

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Medium-size-artery vasculitides do occur in childhood and manifest, in the main, as polyarteritis nodosa (PAN), cutaneous PAN and Kawasaki disease. Of these, PAN is the most serious, with high morbidity and not inconsequential mortality rates. New classification criteria for PAN have been validated that will have value in epidemiological studies and clinical trials. Renal involvement is common and recent therapeutic advances may result in improved treatment options. Cutaneous PAN is a milder disease characterised by periodic exacerbations and often associated with streptococcal infection. There is controversy as to whether this is a separate entity or part of the systemic PAN spectrum. Kawasaki disease is an acute self-limiting systemic vasculitis, the second commonest vasculitis in childhood and the commonest cause of childhood-acquired heart disease. Renal manifestations occur and include tubulointerstitial nephritis and renal failure. An infectious trigger and a genetic predisposition seem likely. Intravenous immunoglobulin (IV-Ig) and aspirin are effective therapeutically, but in resistant cases, either steroid or infliximab have a role. Greater understanding of the pathogenetic mechanisms involved in these three types of vasculitis and better long-term follow-up data will lead to improved therapy and prediction of prognosis.

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Section: Cutaneous Polyarteritis Nodosamentioning

confidence: 99%

Medium-size-vessel vasculitis

Dillon¹,

Eleftheriou²,

Brogan³

2009

Pediatr Nephrol

110

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“…Some cases require administration of systemic corticosteroids 11. Intractable cases require immunosuppression with CYC, azathioprine or methotrexate 12. There is no anecdotal information available on treatment for relapses of CPAN.…”

Section: Discussionmentioning

confidence: 99%

Gangrenous digital infarcts in a severe case of cutaneous polyarteritis nodosa

Mahmood-Rao¹,

Ding

Gandhi

2017

BMJ Case Reports

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Cutaneous polyarteritis nodosa (CPAN) is a rare diagnosis which is distinct from polyarteritis nodosa (PAN). PAN is a medium-vessel vasculitis which can affect multiple organs and classically produces microaneurysms in the vasculature. CPAN is limited to the skin mainly affecting small vessels. There is an absence of microaneurysms in CPAN and it does not affect internal organs. However, the histopathological findings on the skin are similar to PAN. CPAN rarely progresses to PAN but relapses more often. We will illustrate a challenging case of a patient with CPAN who developed gangrenous infarcts despite initial immunosuppressive treatment with high-dose steroids and azathioprine. His treatment had to be escalated to intravenous cyclophosphamide which induced disease remission.

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“…[10] The exact duration of treatment is uncertain. Immunosuppressive agents such as cyclophosphamide, azathioprine, or methotrexate, can been used in cases unresponsive to steroid therapy.…”

Section: Discussionmentioning

confidence: 99%

“…Immunosuppressive agents such as cyclophosphamide, azathioprine, or methotrexate, can been used in cases unresponsive to steroid therapy. [810] The non-steroidal anti-inflammatory (NSAIDs) drugs are used only in the milder form of CPAN, and antibiotics are used in cases of antecedent streptococcal throat infections. In cases resistant to systemic steroids and immunosuppressive therapy, studies have shown that intravenous immunoglobulin can be successfully used.…”

Section: Discussionmentioning

confidence: 99%

Cutaneous polyarteritis nodosa: A rare isolated cutaneous vasculitis

Subbanna

Singh

Swaminathan

2012

Indian Dermatol Online J

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Cutaneous polyarteritis nodosa (CPAN) is a rare form of cutaneous vasculitis that involves small and medium sized arteries of the dermis and subcutaneous tissue without systemic involvement. It presents with tender subcutaneous nodules, digital gangrene, livedo reticularis and subcutaneous ulcerations. The diagnosis is by skin biopsy and characteristic pathologic feature is a leukocytoclastic vasculitis in the small to medium-sized arterioles of the dermis. We report a rare case of benign cutaneous PAN in a 14-year-old girl who presented with history of fever, subcutaneous nodules with cutaneous ulcer and digital gangrene. The skin biopsy showed leukocytoclastic vasculitis with fibrinoid necrosis in the dermal vessels. She received treatment with steroids and lesions resolved completely over a period of month.

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Successful Treatment in Two Cases of Steroid-Dependent Cutaneous Polyarteritis nodosa with Low-Dose Methotrexate

Cited by 38 publications

References 5 publications

Medium-size-vessel vasculitis

Medium-size-vessel vasculitis

Gangrenous digital infarcts in a severe case of cutaneous polyarteritis nodosa

Cutaneous polyarteritis nodosa: A rare isolated cutaneous vasculitis

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