Case of Hydroxyurea-Associated Cutaneous Only Polyarteritis Nodosa

Hydroxyurea is a common antineoplastic agent used for the treatment of several cancers and myeloproliferative conditions, such as chronic myelogenous leukemia and polycythemia vera. Long-term use has been associated with several adverse cutaneous reactions, including skin eruptions, skin ulcers, xerosis, and hyperpigmentation, amongst others. We report a patient who presented with tender, indurated, erythematous nodules in his legs and thighs who had been taking hydroxyurea for 8 years to treat polycythemia vera. Biopsy revealed medium-sized arteries with intramural edema and intramural neutrophils and eosinophils. Histopathology and clinical features were highly suggestive of cutaneous polyarteritis nodosa (CPAN). Discontinuation of hydroxyurea resulted in full resolution of his lesions.

Therapeutic options for cutaneous polyarteritis nodosa: a systematic review

Objective Cutaneous polyarteritis nodosa (CPAN) is a necrotizing vasculitis of the middle-size vessels, confined to the skin. We conducted a systematic review in order to identify studies evaluating the different treatment modalities used in CPAN. Methods This systematic review was conducted according to PRISMA guidelines, registered in PROSPERO: CRD42020222195. PubMed/Medline databases were searched from inception to December of 2020 using the terms: “Polyarteritis nodosa[Title/Abstract]) AND ((therapy[Title/Abstract]) OR (management[Title/Abstract]) OR (treatment[Title/Abstract]))” and “Cutaneous arteritis [Title/Abstract]”. Articles evaluating pertaining to the management of CPAN in adults were eligible for inclusion. Results A total of 7 eligible case series with 325 unique patients were included. No study included a control population. In general, systemic corticosteroids were widely used as induction treatment. Immunosuppressive agents combined with corticosteroids were azathioprine, hydroxychloroquine, sulfasalazine, sulphapyridine, cyclophosphamide, methotrexate, mycophenolate, tacrolimus, rituximab, and thalidomide. Other agents utilized in the studies were dapsone, colchicine, non-steroid anti-inflammatory drugs, salicylates, warfarin and clopidogrel. In some studies, the presence of ulcerations was associated with an increased risk of relapse. Conclusion The evidence available regarding the management of patients with CPAN is limited at best. Further studies are needed in order to evaluate the effect of treatment on disease remission, relapses, and mortality.

Hepatitis B virus infection associated with polyarteritis nodosa and microscopic polyangiitis

We reported a unique case with the coexistence of classic and cutaneous polyarteritis nodosa (PAN), and microscopic polyangiitis (MPA) in hepatitis virus-associated vasculitis. A 77-year-old Asian man presented with extremity weakness and weight loss found to have bilateral foot drop and rash on his hands and legs. Labs reveal positive for hepatitis B core antibody and perinuclear-antineutrophil cytoplasmic antibody (p-ANCA), decreased C3 and C4 levels. Skin biopsy of rash shows medium vessel vasculitis suggesting PAN. Interestingly, renal biopsy showed features of necrotising medium-sized arteritis consistent with PAN and focal crescentic glomerulonephritis consistent with MPA. The patient was treated with 1 g of solumedrol daily for 3 days, followed by oral steroids and cyclophosphamide treatment for vasculitis, and entecavir for chronic hepatitis B infection, resulting in resolution of symptoms. The patient has not had a relapse at 6 months.