Cutaneous polyarteritis nodosa: revisiting its definition and diagnostic criteria

Abstract

Polyarteritis nodosa (PN) is a classical collagen disease with poor prognosis that demonstrates systemic necrotizing vasculitis of small and medium-sized arteries. Cutaneous symptoms are observed in 25-60% of PN patients. On other hand, cutaneous polyarteritis nodosa (CPN) is designated for the cutaneous limited form of PN and demonstrates benign prognosis. However, there has been much debate on whether or not CPN can progress to PN. Although CPN lesions are fundamentally limited to skin, some CPN cases show extracutaneous symptoms such as peripheral neuropathy and myalgia. According to PN diagnostic criteria, which were established by the Ministry of Health, Labour and Welfare of Japan, a disease with both cutaneous and at least one extracutaneous symptom with appropriate histopathological findings can be diagnosed as PN. The same is true according to diagnostic criteria established by the American College of Rheumatology. In addition, there are no specific diagnostic criteria for CPN. In this study, CPN cases were retrospectively collected from multiple Japanese clinics, and analyzed for detailed clinical and histopathological manifestations, in order to redefine the clinical entity of CPN and to propose appropriate diagnostic criteria for CPN and PN. According to the CPN description in Rook's Textbook of Dermatology, we collected 22 cases with appropriate histopathological findings. Of the 22 cases, none progressed to PN or death during the follow-up period, 32% had peripheral neuropathy and 27% had myalgia. Regarding extracutaneous symptoms with CPN, 17 dermatological specialists in vasculitis sustained the opinion that CPN can be accompanied by peripheral neuropathy and myalgia but these symptoms are limited to the same area as skin lesions. Based on these results, we devised new drafts for CPN and PN diagnostic criteria. Our study shows the efficacy of these criteria and most dermatologists recognized that our new diagnostic criteria for CPN and PN are appropriate at the present time. In conclusion, this study suggests that CPN does not progress to PN, and introduces new drafts for CPN and PN diagnostic criteria.

The nonsystemic vasculitic neuropathies

“…Cutaneous polyarteritis nodosa is a necrotizing, nonsystemic skin-nerve vasculitis of small-to-medium-sized arteries in the dermis and hypodermis that manifests with painful nodules, livedo racemosa, ulcers, atrophie blanche, purpura, indurated plaques and necrosis [77][78][79][80][81] . The condition predominates in the lower limbs, but the arms and trunk are also affected in 10-50% patients.…”

“…The mainstays of treatment are short courses of nonsteroidal anti-inflammatories or corticosteroids, but immunosuppressive agents can be used to permit corticosteroid tapering. On the basis of data from numerous reports, 40-45% of patients develop a lower-limb neuropathy, more commonly a multifocal neuropathy than a distal polyneuropathy 78,[80][81][82][83][84][85] . Reports of nerve biopsies in patients with cutaneous polyarteritis nodosa are rare, but one revealed vasculitis 86 .…”

New algorithm (KAWAKAMI algorithm) to diagnose primary cutaneous vasculitis

“…This Japanese study introduced new drafts for CPN diagnostic criteria and described CPN as a distinct clinical entity with benign and chronic courses without systemic involvement. 11 Based on these findings, CPN and polyarteritis nodosa can be considered different entities, whereby CPN does not necessarily progress to polyarteritis nodosa. In CLA, necrotizing vasculitis occurs in small vessels, namely, arterioles, capillaries and venules (also referred to as leukocytoclastic vasculitis because the vascular walls are thin and fibrinoid degeneration occurs in conjunction with collapse of the neutrophils); in addition, it lacks systemic vasculitis and affects only the skin.…”

A case of refractory cutaneous polyarteritis nodosa in a patient with hepatitis B carrier status successfully treated with tumor necrosis factor alpha blockade

“…The flares were characterized by the development of myalgias, purpura, and painful subcutaneous nodes in the legs with elevated CRP levels. During a seven-year clinical course, this case evinced several features consistent with CPAN, namely cutaneous lesions, arthralgias, myalgias, histopathological vasculitis, and absence of hypertension and organ involvement other than peripheral neuropathy [4]. The characteristic cutaneous lesions of CPAN are purpura, livedo reticularis, painful subcutaneous nodules, ulcerations, and infarctions usually arising on the lower extremities [3-5, 8, 11].…”

“…Cutaneous PAN (CPAN) is a chronic and often relapsing cutaneous limited form of necrotizing inflammation involving small or medium-sized arteries without visceral involvement [2]. Some reports suggest that CPAN may represent a distinct subset of classic PAN, and does not progress to PAN [3][4][5]. Several cases of CPAN with HB carrier status have been reported [6,7].…”