Vasculitis restricted to the lower limbs: a clinical and histopathological study

Abstract

Vasculitis of the calf muscles must be considered for patients with calf pain and with a biological inflammatory syndrome.

Four Cases of Polyarteritis Nodosa Presenting Initially as Pain and Pitting Edema in Both Lower Extremities

“…The clinical features of muscular PAN including disabling muscle pain frequently associated with edema in both lower legs and fever were not significantly different from previous reports ( Table 1). The calf is the most frequently involved muscle, but other lower limb areas, particularly the quadriceps, may be involved [7]. The symptoms of muscular PAN can be often confused with vascular or neurogenic claudication or soft tissue infection, as in our series.…”

“…As in our cases, extremities, especially lower limb, can be sole or first affected body parts. In the literature, similar cases have been described under different names such as calf muscle vasculitis, limb restricted vasculitis and muscular PAN [5][6][7]. The most striking feature of muscular PAN is a noticeable paucity of systemic organ manifestations except for cutaneous or testicular involvement [5].…”

“…The most striking feature of muscular PAN is a noticeable paucity of systemic organ manifestations except for cutaneous or testicular involvement [5]. The progression from limited to systemic PAN is rare (under 10%) and extra-musculoskeletal major organ involvement is not observed in other case series of muscular PAN after prolonged follow-up [7]. However, some limited forms might be cases of systemic PAN diagnosed early and treated, as in cases 3 and 4.…”

“…In our series, all patients could be treated effectively without cyclophosphamide therapy, but three of them needed additional MTX therapy to achieve remission or control flare-up. A clinical response to corticosteroids in muscular PAN is excellent within one week, but the relapse rate is highly variable depending on the time course of tapering the corticosteroids [7,12]. Taken together, patients even with FFS of 0 must be informed of the risk of relapse despite initially successful corticosteroid therapy and should be protected from pneumocystis or fungal infection in older ages, as in case 4.…”

Vasculitis of the Central Nervous System – A Rare Cause of Stroke

“…4. ) [12,18,41,42]. Twenty of patients with PAN had brain involvement in the monograph by Schmidley [15].…”