Cutaneous periarteritis nodosa

Díaz‐Pérez, J.L.

doi:10.1001/archderm.110.3.407

Cited by 46 publications

(39 citation statements)

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“…Systemic lupus erythematosus tends not to produce joint destruction except after aseptic necrosis, although weakening of the periarticular tissues in the hands is not uncommon (10,15). The arthritis of cutaneous polyarteritis appears to be nondestructive, as noted by Diaz-Perez and Winkelmann (3) and in the present study. Although they had knee effusions persisting for 20 and 6 years, our patients have not shown clinical or radiologic evidence of joint destruction.…”

Section: Discussionsupporting

confidence: 88%

“…These histopathologic findings are identical with those of the skin lesions of systemic polyarteritis. However the predominant cutaneous findings, usual absence of visceral lesions, and benign course of cutaneous polyarteritis are the basis of its delineation from systemic polyarteritis (1)(2)(3) We have observed a chronic nondestructive arthritis of the knees in 2 patients with cutaneous polyarteritis for 20 and 5 years. This report describes the clinical features, light microscopic findings in the skin, and light and electron microscopic findings in synovial biopsy specimens of this unusual arthritis.…”

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confidence: 91%

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Chronic Nondestructive Arthritis Associated with Cutaneous Polyarteritis

Smukler

Schumacher

1977

Arthritis & Rheumatism

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Two patients with arthritis of the knee joints associated with cutaneous polyarteritis have been followed for 20 and 5 years. The arthritis is characterized by mild to moderate pain and stiffness and inflammatory joint effusions with predominantly polymorphonuclear leukocytes. Despite its chronicity, there has been no clinical or radiologic evidence of joint destruction. Necrotizing inflammation was seen in arteries of the deep skin but not in the small vessels observed in the synovial biopsy specimens.Cutaneous polyarteritis is characterized by a variety of cutaneous lesions, a benign course, and necrotizing arteritis (1-3). The skin findings, which are often localized to the lower extremities, most frequently include painful erythematous nodules, ulcerations, and livedo reticularis. Cutaneous polyarteritis, however, is not strictly a cutaneous disorder, because arthralgia, arthritis and peripheral neuropathy, and fever may also occur. The generally benign course may be brief, protracted over many years, or episodic with spontaneous or corticosteroid-induced remissions (3). In many patients the white blood cell count and/or erythrocyte sedimentation rate is increased. Diagnosis is confirmed by an excision biopsy of the skin deep enough to detect necrotizing inflammation of arteries in the deep dermis or subcutaneous tissue. These histopathologic findings are identical with those of the skin lesions of systemic polyarteritis. However the predominant cutaneous findings, usual absence of visceral lesions, and benign course of cutaneous polyarteritis are the basis of its delineation from systemic polyarteritis (1-3)We have observed a chronic nondestructive arthritis of the knees in 2 patients with cutaneous polyarteritis for 20 and 5 years. This report describes the clinical features, light microscopic findings in the skin, and light and electron microscopic findings in synovial biopsy specimens of this unusual arthritis. Possible reasons for the benign nature of this joint disease are presented. CASE REPORTS Patient AThis 68-year-old white man developed painful tender skin lesions over the legs and ankles at the outset of his illness, which has now persisted for 20 years. Hospital evaluation at onset showed that he was emaciated, febrile, and normotensive. There were multiple hemorrhagic, exquisitely tender, and indurated lesions measuring 1.5 to 2 cm in diameter over the anterior surface of the legs, the malleoli, and the dorsurn of the feet. Except for pitting edema which extended to the knees

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Section: Discussionsupporting

confidence: 88%

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confidence: 91%

Chronic Nondestructive Arthritis Associated with Cutaneous Polyarteritis

Smukler

Schumacher

1977

Arthritis & Rheumatism

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“…Kussmaul and Meier described the first cases of systemic PAN in 1866 [1]. Early reports [6, 54] confirm that cPAN is a separate entity to systemic PAN. We have limited our definition of cPAN to disease affecting the skin, muscle, joints, and peripheral nervous system, with corresponding biopsy confirmation.…”

Section: Discussionmentioning

confidence: 99%

Cutaneous Polyarteritis Nodosa in Childhood: A Case Report and Review of the Literature

Bansal

Houghton

2010

Arthritis

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Polyarteritis nodosa is a rare vasculitis of childhood. Cutaneous PAN (cPAN) is limited to the skin, muscles, joints, and peripheral nerves. We describe a 7.5-year-old girl with cPAN presenting initially as massive cervical edema who later went on to develop subcutaneous nodules, livedo reticularis, myositis, arthritis, and mononeuritis multiplex. The use of corticosteroids resulted in initial clinical improvement, but symptom recurrence necessitated disease modifying antirheumatic drugs and biologic therapy. We review a further 119 reports of biopsy proven cPAN in the literature. A majority of patients (96.6%) had cutaneous involvement; musculoskeletal involvement was common and included both articular (58.0%) and muscular (42.9%) symptoms, and nervous system involvement was least common (18.5%). Corticosteroids were used in the majority of patients (85.7%), followed by use of disease modifying antirheumatic drugs (33.0%), nonsteroidal anti-inflammatory drugs (10.7%), and intravenous immunoglobulin (9.8%). Therapy of cPAN with biologics has only been reported in 2 patients, and we report the first patient treated with Rituximab. A diagnosis of cPAN should be considered in a child with fever, vasculitic rash, and musculoskeletal symptoms. Most children respond to corticosteroids and have a benign course, but some require disease modifying antirheumatic drugs and biologic therapies.

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“…[5][6][7][8][9] While most small studies do not demonstrate any gender predominance among patients with CPAN, a large one found a male : female ratio of 1 : 1.7. [9][10][11] The average age at the time of diagnosis was 43.5 years (range, 6-72) for patients without skin ulceration, and 47 years (range for those with ulcers. 9 Another analysis of 22 patients with CPAN disclosed an age of onset ranging from 17 to 77 years with female patients comprising 86%.…”

Section: Epidemiologymentioning

confidence: 99%

Cutaneous polyarteritis nodosa: a comprehensive review

2010

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Cutaneous polyarteritis nodosa is a rare form of vasculitis relating to small-to-medium-sized arteries. Its etiology is unknown. Clinical manifestations include tender subcutaneous nodules, livedo reticularis, cutaneous ulcers and necrosis. Although it is distinct from systemic polyarteritris nodosa in that it lacks significant internal organ involvement, extra-cutaneous manifestations may be evident. Commonly encountered symptoms include fever, malaise, myalgias, arthralgias, and paresthesias. Exclusion of systemic polyarteritis nodosa is essential in diagnosis. The clinical course is chronic with remissions, relapses, and a favorable prognosis. Mild cases may resolve with nonsteroidal anti-inflammatory drugs. If more severe, treatment with systemic corticosteroids generally achieves adequate response; however, adjunctive therapy is often necessary to allow reduction in steroid dosage.

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Cutaneous periarteritis nodosa

Cited by 46 publications

References 0 publications

Chronic Nondestructive Arthritis Associated with Cutaneous Polyarteritis

Chronic Nondestructive Arthritis Associated with Cutaneous Polyarteritis

Cutaneous Polyarteritis Nodosa in Childhood: A Case Report and Review of the Literature

Cutaneous polyarteritis nodosa: a comprehensive review

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