Cutaneous polyarteritis nodosa
- Robert G Micheletti, MD
Robert G Micheletti, MD
- Assistant Professor of Dermatology and Medicine
- University of Pennsylvania
- Perelman School of Medicine
- Section Editors
- Jeffrey Callen, MD, FACP, FAAD
Jeffrey Callen, MD, FACP, FAAD
- Editor-in-Chief — Dermatology
- Section Editor — Skin and Systemic Disease
- Professor of Medicine
- University of Louisville School of Medicine
- Peter A Merkel, MD, MPH
Peter A Merkel, MD, MPH
- Section Editor — Vasculitis
- Chief, Division of Rheumatology
- University of Pennsylvania
- Deputy Editors
- Abena O Ofori, MD
Abena O Ofori, MD
- Deputy Editor — Dermatology
- Assistant Professor of Medicine, Dermatology Division
- Medical College of Georgia
- Monica Ramirez Curtis, MD, MPH
Monica Ramirez Curtis, MD, MPH
- Deputy Editor — Rheumatology
- Instructor of Medicine, Part-time
- Harvard Medical School
Cutaneous polyarteritis nodosa (cPAN) is a type of vasculitis affecting medium-sized vessels in the skin, a distinct subset of polyarteritis nodosa (PAN) first described in 1931 . Whereas classic systemic PAN commonly affects the kidneys, joints, muscles, nerves, and gastrointestinal tract, as well as the skin, vasculitis in cPAN is skin-limited. (See "Clinical manifestations and diagnosis of polyarteritis nodosa in adults".)
Sometimes called "benign cutaneous PAN," cPAN can nevertheless result in significant morbidity due to painful skin nodules, digital infarcts, ulcerations, and other complications (picture 1A-E). Mild systemic symptoms, which must be distinguished from systemic vasculitis, may also develop.
The cutaneous manifestations of cPAN and systemic PAN are similar; therefore, all patients with cPAN should be evaluated for systemic involvement at the time of diagnosis. In addition, patients with cPAN should be monitored closely over time for progression to systemic PAN. Progression of cPAN to systemic PAN is rare but has been reported.
The clinical features, diagnosis, and treatment of cPAN will be reviewed here. Systemic PAN and the general approach to the diagnosis of vasculitis are reviewed separately. (See "Clinical manifestations and diagnosis of polyarteritis nodosa in adults" and "Treatment and prognosis of polyarteritis nodosa" and "Overview of and approach to the vasculitides in adults" and "Evaluation of adults with cutaneous lesions of vasculitis".)
Polyarteritis nodosa (PAN) is itself a rare disease, with an estimated prevalence of roughly 31 cases per million . cPAN is an even rarer subset of PAN for which the true prevalence is unknown . cPAN may account for approximately 4 percent of PAN cases .
- Lindberg K. Ein Beitrax zur Kenntnis der Periarteritis nodosa. Acta Med Scand 1931; 76:183.
- Mahr A, Guillevin L, Poissonnet M, Aymé S. Prevalences of polyarteritis nodosa, microscopic polyangiitis, Wegener's granulomatosis, and Churg-Strauss syndrome in a French urban multiethnic population in 2000: a capture-recapture estimate. Arthritis Rheum 2004; 51:92.
- Morgan AJ, Schwartz RA. Cutaneous polyarteritis nodosa: a comprehensive review. Int J Dermatol 2010; 49:750.
- Pagnoux C, Seror R, Henegar C, et al. Clinical features and outcomes in 348 patients with polyarteritis nodosa: a systematic retrospective study of patients diagnosed between 1963 and 2005 and entered into the French Vasculitis Study Group Database. Arthritis Rheum 2010; 62:616.
- Daoud MS, Hutton KP, Gibson LE. Cutaneous periarteritis nodosa: a clinicopathological study of 79 cases. Br J Dermatol 1997; 136:706.
- Criado PR, Marques GF, Morita TC, de Carvalho JF. Epidemiological, clinical and laboratory profiles of cutaneous polyarteritis nodosa patients: Report of 22 cases and literature review. Autoimmun Rev 2016; 15:558.
- Nakamura T, Kanazawa N, Ikeda T, et al. Cutaneous polyarteritis nodosa: revisiting its definition and diagnostic criteria. Arch Dermatol Res 2009; 301:117.
- Merlin E, Mouy R, Pereira B, et al. Long-term outcome of children with pediatric-onset cutaneous and visceral polyarteritis nodosa. Joint Bone Spine 2015; 82:251.
- Bansal NK, Houghton KM. Cutaneous polyarteritis nodosa in childhood: a case report and review of the literature. Arthritis 2010; 2010:687547.
- Diaz-Perez JL, Schroeter AL, Winkelmann RK. Cutaneous periarteritis nodosa: immunofluorescence studies. Arch Dermatol 1980; 116:56.
- Kawakami T, Yamazaki M, Mizoguchi M, Soma Y. High titer of anti-phosphatidylserine-prothrombin complex antibodies in patients with cutaneous polyarteritis nodosa. Arthritis Rheum 2007; 57:1507.
- Fathalla BM, Miller L, Brady S, Schaller JG. Cutaneous polyarteritis nodosa in children. J Am Acad Dermatol 2005; 53:724.
- Assicot C, Bourrat E, Prigent F, et al. [Cutaneous polyarteritis nodosa in children: three cases]. Ann Dermatol Venereol 2002; 129:207.
- Bauzá A, España A, Idoate M. Cutaneous polyarteritis nodosa. Br J Dermatol 2002; 146:694.
- Choi SW, Lew S, Cho SD, et al. Cutaneous polyarteritis nodosa presented with digital gangrene: a case report. J Korean Med Sci 2006; 21:371.
- Stein RH, Phelps RG, Sapadin AN. Cutaneous polyarteritis nodosa after streptococcal necrotizing fasciitis. Mt Sinai J Med 2001; 68:336.
- Sheth AP, Olson JC, Esterly NB. Cutaneous polyarteritis nodosa of childhood. J Am Acad Dermatol 1994; 31:561.
- Minkowitz G, Smoller BR, McNutt NS. Benign cutaneous polyarteritis nodosa. Relationship to systemic polyarteritis nodosa and to hepatitis B infection. Arch Dermatol 1991; 127:1520.
- Van de Pette JE, Jarvis JM, Wilton JM, MacDonald DM. Cutaneous periarteritis nodosa. Hepatitis B surface antigen-containing immunocomplexes and polymorphonuclear-leukocyte lysosomal enzyme release. Arch Dermatol 1984; 120:109.
- Whittaker SJ, Dover JS, Greaves MW. Cutaneous polyarteritis nodosa associated with hepatitis B surface antigen. J Am Acad Dermatol 1986; 15:1142.
- Naouri M, Bacq Y, Machet MC, et al. [Interferon-alpha and ribavirin treatment in a patient with hepatitis C virus-associated cutaneous periarteritis nodosa]. Ann Dermatol Venereol 2006; 133:679.
- Durst R, Goldschmidt N, Ben Yehuda A. Parvovirus B19 infection associated with myelosuppression and cutaneous polyarteritis nodosa. Rheumatology (Oxford) 2002; 41:1210.
- Imanishi H, Tsuruta D, Oshimo T, et al. Cutaneous polyarteritis nodosa induced by Mycobacterium tuberculosis. J Dermatol 2012; 39:738.
- Magnant J, Lhommet C, Machet L, et al. [Cutaneous polyarteritis nodosa and Crohn's disease: An association not to be ignored]. Rev Med Interne 2009; 30:345.
- Komatsuda A, Kinoshita K, Togashi M, et al. Cutaneous polyarteritis nodosa in a patient with Crohn's disease. Mod Rheumatol 2008; 18:639.
- Tschetter AJ, Liu V, Wanat KA. Cutaneous polyarteritis nodosa presenting as a solitary blue toe. J Am Acad Dermatol 2014; 71:e95.
- Culver B, Itkin A, Pischel K. Case report and review of minocycline-induced cutaneous polyarteritis nodosa. Arthritis Rheum 2005; 53:468.
- Katada Y, Harada Y, Azuma N, et al. Minocycline-induced vasculitis fulfilling the criteria of polyarteritis nodosa. Mod Rheumatol 2006; 16:256.
- Schrodt BJ, Callen JP. Polyarteritis nodosa attributable to minocycline treatment for acne vulgaris. Pediatrics 1999; 103:503.
- Schaffer JV, Davidson DM, McNiff JM, Bolognia JL. Perinuclear antineutrophilic cytoplasmic antibody-positive cutaneous polyarteritis nodosa associated with minocycline therapy for acne vulgaris. J Am Acad Dermatol 2001; 44:198.
- Pelletier F, Puzenat E, Blanc D, et al. Minocycline-induced cutaneous polyarteritis nodosa with antineutrophil cytoplasmic antibodies. Eur J Dermatol 2003; 13:396.
- Odhav A, Odhav C, Dayal NA. Rare adverse effect of treatment with minocycline. Minocycline-induced cutaneous polyarteritis nodosa. JAMA Pediatr 2014; 168:287.
- Navon Elkan P, Pierce SB, Segel R, et al. Mutant adenosine deaminase 2 in a polyarteritis nodosa vasculopathy. N Engl J Med 2014; 370:921.
- Gonzalez Santiago TM, Zavialov A, Saarela J, et al. Dermatologic Features of ADA2 Deficiency in Cutaneous Polyarteritis Nodosa. JAMA Dermatol 2015; 151:1230.
- Diaz-Perez JL, Winkelmann RK. Cutaneous periarteritis nodosa. Arch Dermatol 1974; 110:407.
- Ha CT, Nousari HC. Surgical pearl: double-trephine punch biopsy technique for sampling subcutaneous tissue. J Am Acad Dermatol 2003; 48:609.
- Ricotti C, Kowalczyk JP, Ghersi M, Nousari CH. The diagnostic yield of histopathologic sampling techniques in PAN-associated cutaneous ulcers. Arch Dermatol 2007; 143:1334.
- Segura S, Pujol RM, Trindade F, Requena L. Vasculitis in erythema induratum of Bazin: a histopathologic study of 101 biopsy specimens from 86 patients. J Am Acad Dermatol 2008; 59:839.
- Chen KR. Cutaneous polyarteritis nodosa: a clinical and histopathological study of 20 cases. J Dermatol 1989; 16:429.
- Rogalski C, Sticherling M. Panarteritis cutanea benigna--an entity limited to the skin or cutaneous presentation of a systemic necrotizing vasculitis? Report of seven cases and review of the literature. Int J Dermatol 2007; 46:817.
- Flanagan N, Casey EB, Watson R, Barnes L. Cutaneous polyarteritis nodosa with seronegative arthritis. Rheumatology (Oxford) 1999; 38:1161.
- Borrie P. Cutaneous polyarteritis nodosa. Br J Dermatol 1972; 87:87.
- Khoo BP, Ng SK. Cutaneous polyarteritis nodosa: a case report and literature review. Ann Acad Med Singapore 1998; 27:868.
- Maillard H, Szczesniak S, Martin L, et al. [Cutaneous periarteritis nodosa: diagnostic and therapeutic aspects of 9 cases]. Ann Dermatol Venereol 1999; 126:125.
- Fink CW. The role of the streptococcus in poststreptococcal reactive arthritis and childhood polyarteritis nodosa. J Rheumatol Suppl 1991; 29:14.
- Boehm I, Bauer R. Low-dose methotrexate controls a severe form of polyarteritis nodosa. Arch Dermatol 2000; 136:167.
- Mimouni D, Ng PP, Rencic A, et al. Cutaneous polyarteritis nodosa in patients presenting with atrophie blanche. Br J Dermatol 2003; 148:789.
- Jorizzo JL, White WL, Wise CM, et al. Low-dose weekly methotrexate for unusual neutrophilic vascular reactions: cutaneous polyarteritis nodosa and Behçet's disease. J Am Acad Dermatol 1991; 24:973.
- Schartz NE, Alaoui S, Vignon-Pennamen MD, et al. Successful treatment in two cases of steroid-dependent cutaneous polyarteritis nodosa with low-dose methotrexate. Dermatology 2001; 203:336.
- Kawakami T, Okano T, Takeuchi S, et al. Complete resolution of refractory cutaneous arteritis by intravenous cyclophosphamide pulse therapy. Int J Dermatol 2015; 54:e323.
- Campanilho-Marques R, Ramos F, Canhão H, Fonseca JE. Remission induced by infliximab in a childhood polyarteritis nodosa refractory to conventional immunosuppression and rituximab. Joint Bone Spine 2014; 81:277.
- Vega Gutierrez J, Rodriguez Prieto MA, Garcia Ruiz JM. Successful treatment of childhood cutaneous polyarteritis nodosa with infliximab. J Eur Acad Dermatol Venereol 2007; 21:570.
- Valor L, Monteagudo I, de la Torre I, et al. Young male patient diagnosed with cutaneous polyarteritis nodosa successfully treated with etanercept. Mod Rheumatol 2014; 24:688.
- Lobo I, Ferreira M, Silva E, et al. Cutaneous polyarteritis nodosa treated with intravenous immunoglobulins. J Eur Acad Dermatol Venereol 2008; 22:880.
- CLINICAL FEATURES
- History and physical examination
- Skin biopsy
- Laboratory tests
- Other tests
- DIFFERENTIAL DIAGNOSIS
- NATURAL HISTORY
- Mild disease
- Acute flares, ulceration, or extracutaneous symptoms
- cPAN associated with streptococcal infection
- Severe or refractory disease
- SOCIETY GUIDELINE LINKS
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS