A 5-year-old Hispanic female presenting for a routine physical examination was registered as a “well child” but appeared ill and complained of abdominal pain.
History Six days earlier, the child had been seen in the emergency department (ED) for a sore throat. Results of a rapid strep test were positive, and she was prescribed amoxicillin. After 3 days, the mother noted a rash on the buttocks; legs; and feet, both of which were swollen. Back in the ED, blood work was done and the antibiotic was stopped. Now, 3 days later, the child continued to complain of sore throat, rash, and abdominal pain that limited her oral intake. There had been no fever since the initial ED visit, and the mother denied witnessing hematuria or hematochezia. The patient's past medical history was significant only for premature birth. Her only medication was acetaminophen for symptom relief. She had no known drug allergies.
Examination Vital signs were as follows: temperature, 36.1°C; heart rate, 108 beats per minute; respiration rate, 21 breaths per minute; and BP, 97/59 mm Hg. The child appeared tired and ill. Examination of the oropharynx was significant for 4+ erythematous tonsils with palatal petechiae. The neck was supple and without nuchal rigidity. Respirations were not labored, lung fields were clear, and the heart had a regular rate and rhythm with no murmur. Bowel sounds were heard in all four quadrants, and the abdomen was without distention or masses. The patient did not grimace on abdominal examination, nor did she demonstrate guarding or rebound tenderness. Erythematous, nonblanching, circumferential macules ranging in size from 2 or 3 mm to 1 cm were scattered over her buttocks and bilateral lower extremities. The dorsal surfaces of both feet were covered with pinpoint, erythematous, nonblanching lesions that coalesced on the left foot in two areas, each measuring 3 to 4 cm; no joint swelling was appreciated.
Laboratory results CBC revealed a hemoglobin of 13.9 g/dL; WBC count, 11,400/μL with a slightly elevated neutrophil count; and platelet count, 251,000/μL. Urinalysis showed only trace blood.
WHAT IS YOUR DIAGNOSIS?
- Scarlet fever
- Idiopathic thrombocytopenia
- Henoch-Schönlein purpura
- Drug rash
The diagnosis was Henoch-Schönlein purpura (HSP). The most common systemic vasculitis of childhood,1 HSP is presumed to be immune-mediated.2 Although HSP can affect adults, most cases occur in children aged 4 to 7 years, with slightly more males affected than females.2 The exact cause is unknown, although triggers may include viral or bacterial infections.3
The illness occurs most often in the spring.2 HSP characteristically manifests with purpura on the buttocks and lower extremities.2 Because it is a vasculitis and not a coagulopathy, the platelet count is normal.2 More than half of patients report abdominal pain; bloody stool or obstruction can occur concomitantly.3 Joint pain, particularly in the knees and ankles, also occurs in more than half of patients, and joint swelling may be appreciated on examination.3 Blood in the urine or hypertension3 is a less common finding and may signal glomerulonephritis. 2 The diagnosis is primarily a clinical one, although urinalysis should be performed on all patients. A basic metabolic panel should be added for those with renal involvement,3 and additional blood work may be indicated to exclude other diagnoses.3
Treatment is primarily aimed at alleviating symptoms.3 Corticosteroids may be given to alleviate abdominal and joint symptoms,2 although studies of effectiveness have shown mixed results.1 Nearly one-half of patients experience a second episode of HSP, but most patients recover fully with no lasting kidney disease.2
Treatment Since this child was not dehydrated or in severe pain, she was initially given IM penicillin G and treated as an outpatient with acetaminophen. Her mother was advised to push oral fluids and to return for follow-up in 1 week or sooner if the child's symptoms worsened. By the next day, the pain had spread to the patient's feet and hands, with swelling of the hands, and the abdominal pain was continuing. The patient was refusing oral fluids and had minimal urine output. She was immediately admitted for IV hydration and control of pain that limited range of motion of all joints. Edema was apparent in her knees and wrists. Repeat urinalysis showed 1+ protein with few RBCs. She responded well to NSAIDs, hydroxyzine, and prednisone at a dose of 2 mg/kg, with less pain and joint swelling, and was discharged on day 4.
On initial follow-up in the rheumatology clinic, new skin lesions were seen, so prednisone was continued. Two months later, the rash, abdominal and hand pain, and morning stiffness recurred. Amoxicillin was restarted and prednisone continued for 2 more weeks. All her medications have now been tapered to a final dose, and results of a recent urinalysis were normal. JAAPA
1. Weiss PF, Feinstein JA, Luan X, et al. Effects of corticosteroid on Henoch-Schönlein purpura: a systematic review. Pediatrics.
2. Langford CA, Fauci AS. The vasculitis syndromes. In: Longo DL, Fauci AS, Kasper DL, et al, eds. Harrison's Principles of Internal Medicine.
18th ed. New York, NY: McGraw-Hill; 2012: chap 326.
3. Kharbanda AB, Sawaya RD. Acute abdominal pain in children. In: Tintinalli JE, Stapczynski JS, Ma OJ, et al, eds. Tintinalli's Emergency Medicine: A Comprehensive Study Guide.
7th ed. New York, NY: McGraw-Hill; 2011: chap 124.