Cutaneous necrotizing eosinophilic vasculitis is a recently identified type of vasculitis that is characterized by an eosinophil-predominant necrotizing vasculitis affecting small dermal vessels. Clinically, it presents with pruritic erythematous and purpuric papules and plaques, peripheral eosinophilia and a good response to systemic steroid therapy. This vasculitis can be idiopathic or associated with connective tissue diseases. Although the pathogenic roles of eosinophil-derived granule proteins and interleukins have been documented in diseases associated with eosinophilia, a role of CD40 (a glycoprotein of the tumour necrosis factor receptor superfamily) has rarely been described. We describe two patients with idiopathic hypereosinophilic syndrome (HES) presenting with multiple erythematous patches and plaques on the lower extremities and Raynaud's phenomenon. They satisfied the criteria for the diagnosis of HES by clinical and laboratory investigations. Histopathology of the cutaneous lesions revealed prominent eosinophilic infiltration with local fibrinoid change in vessel walls in the dermis and subcutis. Immunohistochemical detection of CD3, CD4, CD8 and CD40 was performed. Infiltrating eosinophils were strongly stained by anti-CD40 monoclonal antibody. One patient improved with prednisolone, pentoxifylline and nifedipine, without recurrence. The other patient initially improved with steroids, but after self-withdrawal of steroid developed digital ischaemia that evolved to severe necrosis and required amputation. Cutaneous necrotizing eosinophilic vasculitis, Raynaud's phenomenon and digital gangrene may develop as cutaneous manifestations of HES. CD40 may play a part in the pathogenesis of eosinophilic vasculitis in HES.

No abstract

Recently, percutaneous epidural neuroplasty has become widely used to treat radicular pain caused by spinal stenosis or a herniated intervertebral disc. A 19-year-old female patient suffering from left radicular pain caused by an L4-L5 intervertebral disc herniation underwent percutaneous epidural neuroplasty of the left L5 nerve root using a Racz catheter. After the procedure, the patient complained of acute motor weakness in the right lower leg, on the opposite site to where the neuroplasty was conducted. Emergency surgery was performed, and swelling of the right L5 nerve root was discovered. The patient recovered her motor and sensory functions immediately after the surgery. Theoretically, the injection of a large volume of fluid in a patient with severe spinal stenosis during epidural neuroplasty can increase the pressure on the opposite side of the epidural space, which may cause injury of the opposite nerve by barotrauma from a closed compartment. Practitioners should be aware of this potential complication.

Polyarteritis nodosa (PAN) has a broad spectrum of clinical presentation, since it affects small and medium-sized muscular arteries with microaneurysm formation, aneurysmal rupture with hemorrhage, thrombosis, and, consequently, organ ischemia or infarction. Although skeletal muscle involvement is well documented in patients with PAN, it can mimic more common diseases, and cause confusion and delays in diagnosis. PAN muscular involvement may have limited or early systemic forms with a benign course and excellent clinical response to corticosteroid therapy. Herein, we describe the clinical course and outcome of four unusual cases of PAN manifested by acute onset of pain and pitting edema in both lower extremities; in addition, we reviewed the relevant literature. (J Rheum Dis 2017;24:48-54)

No abstract