Stroke and skin rash: A rare case of Henoch-Schonlein purpura
A 46-year-old lady was admitted for sudden onset of weakness of the right side of the body and facial deviation without any history of unconsciousness, headache, vomiting or seizure. She was diagnosed as a case of hypertension 15 days back, for which she was given metoprolol 25 mg/day. In the emergency room, examination revealed stable vitals, blood pressure 170/110 mmHg, Glasgow coma score 12, right-sided hemiparesis and left-sided upper motor neuron facial nerve palsy. She also had palpable purpura over both the shins and buttocks and a digital infarct of the left thumb. Systemic examination was otherwise normal.\nThe patient had developed swelling and redness of both the knees and ankles 3 weeks prior to admission, which was associated with severe abdominal pain. She was prescribed corticosteroids for 1 week, which she stopped after relief of symptoms. Soon after withdrawal of the drug, she developed gradual darkening of the tip of the left thumb and painful crops of reddish lesions appeared over her legs and buttocks [].\nOn admission, she underwent routine hematologic and biochemical testing and a noncontrast computed tomography (CT) scan of the brain. There was normochromic normocytic anemia, leukocytosis, raised erythrocyte sedimentation rate (ESR) and normal platelet count []. Her blood urea nitrogen and creatinine was mildly elevated, while other biochemical parameters remained within normal limits. CT brain showed multiple infarcts in the left internal capsule, basal ganglia and right thalamus []. A routine urinalysis revealed presence of albumin and active urine sediment of red blood cells (RBC) and RBC casts. Estimation of 24-h urinary protein showed nonnephrotic range proteinuria []. Ultrasonography of the kidneys showed normal kidney size with increased cortico-medullary echogenecity. Tests for antinuclear factor, anti-neutrophil cytoplasmic antibody (p- and c-ANCA ) and anti-phospholipid antibody were negative. A punch biopsy was taken from the skin lesions over the legs, which revealed leucocytoclastic vasculitis [].\nThe patient was started on low-dose aspirin, metoprolol and ramipril. She remained conscious, oriented and afebrile, without new neurological deficits, and maintained normal urine output. However, on the fourth day of hospital stay, there was onset of abdominal pain and inflammation of both knee joints.\nThe clinical features and biopsy evidence supported the diagnosis of HSP, although age and presentation of the patient were not typical of the disease. Considering the presence of both renal and cerebrovascular involvement, the patient was administered intravenous cyclophosphamide 500 mg/sq meter on Day 1 combined with methylprednisolone 1 gm/day for Days 1–3. This was followed by a maintenance dose of prednisolone 0.5 mg/kg/day.\nThe patient was readmitted after 1 month for follow-up and a repeat pulse of cyclophosphamide and methylprednisolone. On clinical examination, her paresis had improved. Routine investigations of blood and urine showed an improvement of anemia and absence of active urinary sediment. She is scheduled to receive four more monthly pulses of cyclophosphamide and methylprednisolone along with maintenance dose of prednisolone.