Fulminant skin necrosis and massive deep venous thrombosis as a part of a clinical presentation of polyarteritis nodosa -case report

Methods We report the case of 8 year old boy who was referred to our hospital with 11 days history of high fever (39.5°C), fatigue, anorexia and weight loss. Two days prior to admission he began to complain of pain in the calves muscles, developed petechial skin rash and hematomas, gross haematuria and gastrointestinal (GI) bleeding. At admission was febrile, in very poor condition, hyperventilating, with calf muscle tenderness (mostly gastrocnemius and soleus muscles), scrotal pain, unable to walk. Skin findings: livedo reticularis, multiple hematomas, petechie, subcutaneous nodules. On the second day he developed cutaneous necrosis, five days after admission bilateral legs edema was observed. Doppler ultrasonography showed massive deep end superficial venous thrombosis. He was tested negative for hepatitis C antibodies, perinuclear antineutrophilic cytoplasmic antibody (pANCA) and cytoplasmic antineutrophilic cytoplasmic antibody (cANCA). A deep skin biopsy was performed and showed fibrinoid necrosis of small muscular arteries with leukocytic infiltrate; finding compatible with polyarteritis nodosa. Kidney biopsy was contraindicated because the boy had agenesis of the right kidney.