Recurrent Abdominal Pain in a Boy With Delayed Appearance of Palpable Purpura

AFFILIATIONS:
¹Resident Physician, Pediatrics Department, Nemours Children’s Health, Orlando, Florida
²Nephrology Department, Nemours Children’s Health, Orlando, Florida
³Pediatrics Department, Nemours Children’s Health, Orlando, Florida

CITATION:
Cooper F, Mathews N, Kopsombut G. Recurrent abdominal pain in a boy with delayed appearance of palpable purpura. Consultant. 2022;62(12):e9. doi:10.25270/con.2022.08.000005

Received February 22, 2022. Accepted April 28, 2022. Published online August 8, 2022.

DISCLOSURES:
The authors report no relevant financial relationships.

CORRESPONDENCE:
Gift Kopsombut, MD, Nemours Children’s Health Orlando, 6535 Nemours Parkway, Orlando, FL 32827 (Gift.Kopsombut@nemours.org)

A previously healthy 9-year-old boy developed acute upper left quadrant and epigastric abdominal pain, nonbloody nonbilious vomiting, and decreased oral intake. The following day, his pain and symptoms worsened after an accidental fall over his bicycle handlebars. He was then taken to the emergency department (ED) by his parents.

Diagnostic testing. At the ED, a complete blood cell count, comprehensive metabolic panel, and lipase test were conducted, results of which were unremarkable. Results of a urinalysis were within normal limits, except for a moderate level of ketones.

An ultrasonography scan showed a nonincarcerated inguinal hernia, and an abdominal radiograph showed moderate stool burden. A computed tomography (CT) scan with contrast was unremarkable. Constipation was diagnosed. He failed to eat or drink without vomiting and was subsequently admitted to the hospital for further workup.

During admission, he was treated with famotidine, polyethylene glycol, a sodium phosphate enema, and intravenous hydration until oral intake improved and he was discharged home.

He returned to the ED 1 week later with episodic, recurrent abdominal pain. He had an unremarkable clinical examination at that time, with the ability to hydrate, and was discharged home with a diagnosis of duodenal hematoma. He was advised to take acetaminophen at home and follow-up with a gastroenterologist.

About 1 week later, the patient presented again to the ED and was readmitted for recurrent, prolonged constipation, vomiting, abdominal pain, poor oral intake, and a 1.8-kg weight loss. A repeat CT with contrast of the abdomen and pelvis showed a thickened gastric wall with no active inflammation of the small or large bowel. An endoscopy was performed that showed erythema of the stomach and duodenum. Biopsies were conducted, results of which showed patchy gastritis and duodenitis. He was treated with sucralfate, omeprazole, and famotidine as a home regimen. He was observed for 3 days with good pain control and oral food intake prior to discharge.

About 1 week later, the patient presented again to the ED and was readmitted for recurrent abdominal pain and now a cumulative 3.6-kg weight loss. His lipase level was elevated at 315 U/L upon admission but down trended the next day. An ultrasonography scan of the right upper quadrant was conducted because of possible pancreatitis, but results were unremarkable. The patient was treated with morphine initially for pain and was given a bisphosphate enema. His abdominal pain and appetite rapidly improved on cyproheptadine, hyoscyamine, and omeprazole, and he was discharged 1 day later with a normal physical examination and subjective improvement according to the patient and his family.

Two days later, the patient again presented to the ED with sudden onset of acute abdominal pain that occurred after eating a large meal and was unresolved with acetaminophen, polyethylene glycol, and a bisphosphate enema at home. An ultrasonography scan revealed trace peripancreatic free fluid (Figure 1). An abdominal radiograph showed air fluid levels on the upright film. Upon admission to the hospital, he was started on cyproheptadine, hyoscyamine, omeprazole, docusate sodium, and a lactose-free diet. A magnetic resonance-enterography scan showed wall thickening of the small bowel loops. A fecal occult blood test returned positive results. A nasogastric tube was placed for polyethylene glycol cleanout and nutrition. An upper gastrointestinal endoscopy and colonoscopy were performed, showing mild mucosal edema of the second part of the duodenum. A capsule endoscopy was also performed, revealing mild focal patchy erythema and superficial ulceration in the mid small intestine.

Figure 1. Peripancreatic free fluid seen on ultrasound of the abdomen. 

Unfortunately, his abdominal pain was aggravated with the introduction of a full diet. The hospital medicine team, consulting gastroenterologist, and consulting surgeon observed severe episodic pain. An exploratory laparotomy was performed, and a full thickness biopsy of the jejunum was taken, showing normal results. A double lumen tube was placed on low intermittent suction for bilious secretions, and a peripherally inserted central catheter was placed for total parenteral nutrition.

Two weeks into this admission and 6 weeks since the onset of symptoms, the patient developed a palpable purpuric rash on his arms, buttocks, legs, and feet (Figures 2-4). Results of a repeat spot urine sample revealed a protein/creatinine ratio of 8 mg/mg, indicating proteinuria,  and microscopic hematuria. He maintained normal renal function. His clinical diagnosis was immunoglobulin A vasculitis nephritis, for which he was started on methylprednisolone, 2 mg/kg/d. His symptoms rapidly improved, and he was discharged on a 4-week corticosteroid taper.

Figure 2. Purpuric rash on the patient’s bilateral feet.

Figure 3. Purpuric rash on the patient’s right arm surrounding his peripherally inserted central catheter.

Figure 4. Purpuric rash on the patient’s lower back.

Differential diagnosis. The differential diagnosis for chronic abdominal pain in a school-aged child is broad (Table).^1,2 Mild acute injury, constipation, and functional abdominal pain are common causes of abdominal pain in this age group and are initially high in the suspected differential. However, our patient developed red flags for abdominal pain that warranted further investigation, including severe pain requiring narcotics, vomiting, and weight loss. According to both the European and the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition, other alarming symptoms in the presence of constipation include fever, blood in the stool, decreased lower extremity reflexes, and decreased strength.³

Gastroesophageal reflux disease, gastritis, and gastric ulcers were considered, but the patient’s pain persisted despite use of sucralfate, famotidine, and omeprazole. Severe gastritis was reconsidered after the patient’s pain did not resolve despite several weeks of acid-blocking therapy.

The patient then underwent extensive workup for celiac disease, intussusception, inflammatory bowel disease, intrabdominal injury with suspicion of duodenal hematoma, and appendicitis. Gastric and intestinal biopsy results revealed uncomplicated gastritis and duodenitis—ruling out celiac and inflammatory bowel diseases. An immunohistochemical stain for Helicobacter pylori in his biopsy samples was also negative. The patient had no other systemic symptoms, including growth failure or joint aches.

The episodic nature of the patient’s abdominal pain was concerning for intussusception, but several abdominal ultrasonography scans during the patient’s pain episodes were all unremarkable. Abdominal imaging also ruled out gallstones, pancreatitis, pancreatic pseudocysts, renal stones, and abscesses. Although the patient's pain intensified after he fell forward over bicycle handlebars, abdominal imaging and exploratory laparotomy did not reveal a duodenal hematoma.

Discussion. Immunoglobulin A (IgA) vasculitis, also known as Henoch-Schönlein purpura, is a vasculitis seen most often in children aged 5 to 10 years, with a male predominance.⁴ There is no definitive laboratory test that can diagnose IgA vasculitis. The 2010 EULAR/PRINTO/PRES consensus criteria list palpable purpuras not due to thrombocytopenia as required for diagnosis as well as one of the following: abdominal pain, IgA deposition seen in biopsy of the skin, kidney, or small blood vessels, arthritis or arthralgia, or renal involvement.⁵ About 50% to 80% of children exhibit gastrointestinal involvement.⁴ Our patient’s gastrointestinal symptoms were thought to be related to submucosal hemorrhage, edema, and bowel ischemia, which can appear as abdominal pain (that may become worse after eating), nausea, vomiting, hematemesis, melena, or hematochezia.^4,6 Imaging of the bowel may show mural thickness, bowel edema, or hematomas, and endoscopy often shows erosions suggesting gastritis or duodenitis.⁶ Intussusception has also been associated with IgA vasculitis.⁶

In most cases of IgA vasculitis in children, the abdominal pain usually occurs concurrently with the appearance of the purpuric rash. However, there are case reports of abdominal pain preceding skin findings in approximately 5.4% to 17.0% of patients.^4,7,8 Sharieff and colleagues⁹ presented 2 cases: one of a 7.5-year-old and one of a 5-year-old, both of whom presented with abdominal pain before the appearance of a rash (7 days and 9 days, respectively). Kang and colleagues⁸ presented the case of a 15-year-old who had an 11-day history of abdominal pain, vomiting, and decreased appetite before palpable purpuras were seen on her feet. Jarasvaraparn and colleagues⁷ presented the case of a 5-year-old patient with severe abdominal pain, duodenitis on endoscopy, and renal involvement, but no presentation of purpura. They noted that if abdominal pain was the presenting symptom of IgA vasculitis, it had occurred 3 to 30 days before the rash. Therefore, it is surprising that our patient’s abdominal pain persisted for 6 weeks before his purpura appeared.

Treatment and management. Typically, IgA vasculitis is self-limited and requires only supportive care with acetaminophen and nonsteroidal anti-inflammatory drugs. Typical symptoms such as palpable purpura, abdominal pain, and joint pain usually resolve in 4 weeks.¹⁰ Urinalysis should be collected at the time of diagnosis to determine whether there is renal involvement, with referral to a nephrologist if present. Since renal involvement may develop later (up to 6 months postdiagnosis), urinalysis should continue once a week for the first 2 months, then every 2 weeks for months 3 and 4, and then once a month for months 5 and 6.

In patients with severe presentations of abdominal or joint pain that limits daily activities, many case reports in the literature suggest administering corticosteroids for symptomatic relief: prednisolone, 1 mg/kg/d¹¹; methylprednisolone, 2 mg/kg/d⁷; prednisone, 1 to 2 mg/kg/d^8,9; and, in some cases when symptoms persist, intravenous immunoglobulin.⁸ Upon initiation of corticosteroids, resolution has been reportedly seen within 24 hours.¹² If corticosteroids are started, tapering over 3 or 4 weeks to minimize the chance of rebound symptoms should be considered.¹³ However, other health care providers may suggest a shorter course.

Up to one-third of patients with IgA vasculitis may have a subsequent episode, usually within 4 months from initial presentation. This is more commonly seen in patients with severe abdominal or joint involvement. Recurrent episodes are typically less severe.¹⁴

Patient outcome. The patient underwent laboratory tests 3 weeks after hospital discharge that showed subnephrotic range proteinuria (1.4 mg/mg) with resolution of microscopic hematuria. Meanwhile, pending laboratory tests obtained during admission returned negative results for antimyeloperoxidase antibody, C-ANCA, P-ANCA, atypical ANCA, and dsDNA. The patient had an elevated positive proteinase 3 antibody (7.7 U/mL; reference, 0.0-3.5 U/mL) of unknown significance. The corticosteroids were tapered and stopped 4 weeks after hospital discharge.

At the 6-week follow-up visit, the family reported that the patient’s abdominal pain and purpura had completely resolved. The patient reported some intermittent pain in his knees, ankles, and shins. Results of a first-morning urinalysis showed further improvement in proteinuria (0.7 mg/mg) without hematuria. The current plan includes follow-up with a nephrologist every 1 to 2 months and renal biopsy if his symptoms or proteinuria return.

Conclusion. Although abdominal pain is commonly seen as a manifestation of IgA vasculitis, this is the first report of abdominal pain preceding purpura by 6 weeks. IgA vasculitis with abdominal pain preceding the rash is less common but still important in the differential diagnosis of acute, recurrent pediatric abdominal pain.