Chronic Nondestructive Arthritis Associated with Cutaneous Polyarteritis

Abstract

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

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

“…Arthralgia was noted to be quite common in patients with CPN (7) and was also found in 9 of our 20 patients. Despite many episodes of recurrent arthralgias in the clinical course, as has been emphasized by some previous literature (7,32,33), there has been no clinical or radiologic evidence ofjoint destruction in this study. Although no synovial biopsies were performed in this study, synovial biopsies, including open ones, performed by others have not revealed any evidence of necrotizing vasculitis in the synovium (32,33).…”

Development of Arthritis as the Initial Involvement in Adult-Onset Cutaneous Polyarteritis Nodosa: Two Cases and Literature Review

“…Second, arthritis develops as synovitis based on necrotizing vasculitis, but biopsied synovial specimens may be obtained from outside the target site involving typical pathological findings. It was suggested that deep and/or surrounding portions of involved cutaneous or articular lesions may include the definitive histopathological findings [ 6 ]; in fact, our patient's results demonstrated necrotizing vasculitis in the deep dermis, ultimately allowing for the diagnosis of c-PAN. The classification of ANCA-associated vasculitis may be satisfiable in accordance with the criteria of the Chapel Hill Consensus Conference and/or the consensus algorithm proposed by the European Medicines Agency [ 5 , 23 , 24 ]; nevertheless, the definitive diagnosis for both c-PAN and classical PAN necessarily requires histopathological evidence [ 5 , 11 , 23 ].…”

“…Arthralgia is a common clinical feature in PAN, and approximately 40–60% of patients present with articular symptoms [ 2 , 13 , 14 ]. However, to the best of our knowledge, only six cases were found in the English literature when we focused on arthritis as the initial clinical episode of PAN [ 6 – 10 ] ( Table 1 ). Accordingly, arthritis may uncommonly occur as the initial manifestation in adult PAN, although it has been described that arthritis develops as the first symptomatic episode of childhood PAN in 7.7% of patients [ 15 ].…”

“…Accordingly, it was suggested that the long-term persistence of disease activity might lead to the destruction of the joint. Meanwhile, one patient in the reviewed cases presented with nondestructive arthritis although it took 7 years for the initiation of the proper treatment for him [ 6 ]. In addition, a previous case of c-PAN also demonstrated the absence of joint destruction even though the patient had rapidly progressive and repeated relapse of arthritis [ 19 ].…”

“…The clinical findings in a total of eight patients including the six reviewed cases [ 6 – 10 ] and our two cases are summarized in Table 1 . All eight patients were categorized as having c-PAN according to the criteria previously proposed [ 11 , 12 ].…”