Cutaneous Polyarteritis Nodosa: A Clinical and Histopathological Study of 20 Cases

Chen, Ko‐Ron

doi:10.1111/j.1346-8138.1989.tb01582.x

Cited by 122 publications

(113 citation statements)

References 37 publications

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“…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 .…”

Section: Diabetic Lumbosacral Radiculoplexus Neuropathymentioning

confidence: 99%

The nonsystemic vasculitic neuropathies

2017

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The vasculitides are a clinically diverse group of diseases with the histopathological signature of blood vessel-centred inflammation that results in vascular damage and ischaemic injury to the affected tissues 1 . Vasculitis can be caused by drugs, infections and cancers, but diagnostic workup reveals no trigger in most patients. Autoimmune mechanisms are active in all vasculitides, except those caused by direct infection of vessel walls. Most vasculitides are systemic and involve multiple organs and tissues. Those affecting small-to medium-sized vessels often manifest with a vasculitic neuropathy. For example, neuropathies occur in 60-70% of patients with polyarteritis nodosa, eosinophilic granulomatosis with polyangiitis and cryoglobulinaemic vasculitis, and 40-50% of those with microscopic polyangiitis and rheumatoid vasculitis 2 .Vasculitic neuropathy can also occur without systemic vasculitis. This single-organ vasculitis of the PNS has commonly been referred to as nonsystemic vasculitic neuropathy (NSVN), but other forms of clinically isolated PNS vasculitis are now recognized and can be considered variants of NSVN 3 . Although NSVN is classically an acute, relapsing, multifocal neuropathy, it can present as a slowly progressive neuropathy without distinct asymmetries. As such, it should be considered as a possible cause of any progressive axonal neur opathy. NSVN can be diagnosed only with a nerve biopsy, with or without a concomitant muscle or skin biopsy; as nerve biopsies are seldom performed, the condition is under-recognized. The disorder must be distinguished from many other causes of multifocal neuropathy, the definition of which is itself not standardized and requires clarification.A Peripheral Nerve Society guideline group reviewed the literature on vasculitic neuropathy and published consensus recommendations on the classification, diagnosis and immunosuppressive treatment of NSVN in 2010 (REF. 4) Abstract | Nonsystemic vasculitic neuropathy (NSVN) is an under-recognized single-organ vasculitis of peripheral nerves that can only be diagnosed with a nerve biopsy. A Peripheral Nerve Society guideline group published consensus recommendations on the classification, diagnosis and treatment of NSVN in 2010, and new diagnostic criteria for vasculitic neuropathy were developed by the Brighton Collaboration in 2015. In this Review, we provide an update on the classification, diagnosis and treatment of NSVN. NSVN subtypes include Wartenberg migratory sensory neuropathy and postsurgical inflammatory neuropathy. Variants include diabetic radiculoplexus neuropathy and -arguably -neuralgic amyotrophy. NSVN with proximal involvement is sometimes termed nondiabetic lumbosacral radiculoplexus neuropathy. Cutaneous polyarteritis nodosa and other skin-nerve vasculitides overlap with NSVN clinically. Three patterns of involvement in NSVN have been identified: multifocal neuropathy, distal symmetric polyneuropathy, and overlapping multifocal neuropathy (asymmetric polyneuropathy). These patterns lack standard defi...

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Section: Diabetic Lumbosacral Radiculoplexus Neuropathymentioning

confidence: 99%

The nonsystemic vasculitic neuropathies

2017

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“…Thus, CPN and PN can be considered to be two independent diseases. However, since rare cases showing progression to PN during long-term follow up have also been reported, 11) careful observation of the course is necessary. Recently, Kawakami et al reported the presence of an antibody against the CPN-specific phosphatidylserine-prothrombin complex.…”

Section: Independence Of Diseasementioning

confidence: 99%

Cutaneous Polyarteritis Nodosa: An Update

Furukawa

2012

Annals of Vascular Diseases

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Cutaneous symptoms are observed in 25%-60% of polyarteritis nodosa (PN) patients. On the 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, 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 American College of Rheumatology (ACR). 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, one of global standard textbooks, 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.

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“…Studies of c-PAN report fibrinoid necrosis of the vessel wall and infiltration of lymphocytes and monocytes in the vessel lumen and wall. 7,8 Direct immunofluorescence findings are variable and do not distinguish between c-PAN and systemic PAN. 4,9 While the cause of c-PAN is unknown, it has been associated with group A β-hemolytic Streptococcus, parvovirus B19, human immunodeficiency virus, and hepatitis B infections.…”

Section: Discussionmentioning

confidence: 97%

“…2,9 There are, however, rare cases of progression of c-PAN to systemic PAN. 7 For patients with mild disease, nonsteroidal anti-inflammatory drugs or colchicine may be used. 2,4 Patients with refractory disease, severe cutaneous disease, or extracutaneous involvement require more aggressive treatment.…”

Section: Discussionmentioning

confidence: 99%