A Case Of Juvenile Polyarteritis Nodosa With Intestinal Hemorrhage And Multiple Cranial Nerve Palsy

摘要

Polyartheritis Nodosa PAN is not common in adolescents. Here, we report a case of a 13 year old girl with PAN who presented with recurrent abdominal pains followed by cutaneous and peripheral nerves involvements during three years with a relapsing-remitting course. This is a classic case of PAN in an adolescent who diagnosed late in the course of her disease because its monosymptomatic and unusual presentation. Also there are some reports about cranial nerves involvements with separate mechanism. In our case oculomotor and vestibuloacoustic nerves were seen in the absence of signs of brainstem disease. Introduction Kussmaul and Maier first described polyarteritis nodosa (PAN) in 1866 after observing areas of focal inflammatory exudations that gave rise to palpable nodules in a patient with advanced disease (1). PAN, like other vasculitides, is generally multisystem and most commonly affects skin, joints, peripheral nerves, the gut, and the kidney vasculature. In 1948, Davson et al described a microscopic PAN characterized by a diffuse necrotizing glomerulonephritis with similar clinical features to classic PAN but with the additional manifestations of small vessel involvement resulting in rapidly progressive glomerulonephritis (RPGN) and pulmonary capillaritis (2). Polyarteritis nodosa (PAN) is a vasculitis of medium-sized or small vessels. The vasculitis of PAN has a predilection for medium-sized arteries of the skin, peripheral nerves, the gut, and the kidney vasculature. PAN is predominantly observed in patients aged 40-60 years, but can occur in all age groups. When PAN presents in an atypical location and atypical age group, the diagnosis becomes less straightforward.PAN is not common in adolescents. Here, we report a case of a 13 year old girl with PAN who presented with recurrent abdominal pains followed by cutaneous and peripheral nerves involvements during three years with a relapsing-remitting course. This is a classic case of PAN in an adolescent who diagnosed late in the course of her disease because its monosymptomatic and unusual presentation. Case Report In November 2004, a 15-year old girl was admitted to our hospital because of foot drop and abdominal pain.The patient had been well until three years before admission, when recurrent abdominal pains developed. The location of abdominal pains was periumbelical and there was not accompanying nausea and/or womiting, night sweats, intermittent diarrhea and fever. Two years before admission, she noted erythematous vesicles and papules over the both shins with next central ulceration. During last year, she had high blood pressures in her visits in the range of 140/90 to 160/110 mmHg. Some laboratory and radiologic tests were done for evaluation of abdominal pains and hypertention. Immunologic tests for rheumatoid factor, antinuclear antibodies, anti dsDNA antibodies, cANCA, anticadiolipin antibodies, C3, C4, CH50 levels and hepatitis B virus surface antigen and HCV Ab were negative. Erythrocyte sedimentation rate was 8 mm/h and C-reactive protein was 3+ and all other hematological and biochemical tests were normal. Ultrasound examination of abdomen and pelvis, upper GI series, endoscope study of esophagus, stomach and duodenum showed no abnormality. Abdominal and pelvis CT with contrast were normal. Gastric biopsy samples showed chronic inflammatory gastritis. Four month before admission she complained headache, vertigo and diplopia. Third cranial nerve palsy with petosis was diagnosed. Hearing loss in left ear was developed in next days. Magnetic resonance imaging (MRI) was normal. Audiometry showed sensory hearing loss in left ear. Two month before, she had an exacerbation of abdominal pain and erythematous nodules on anterior surface of her legs. So she admitted in other hospital. A skin-biopsy specimen disclosed leukocytoclastic vasculitis and the patient were treated with oral prednisone at a dose of 40 mg daily. Five days before admission, numbness developed in