A 7 year old male presents to your emergency department with a chief complaint of abdominal pain. The patient was seen just five days ago and was evaluated for appendicitis. When you talk to the parents, they state that he has continued to have abdominal pain since the last visit and noticed the development of a rash on his upper and lower extremities. The mother is particularly frightened about the rash because of how quickly it appeared, however the rash does not seem bothersome to the patient. It is not pruritic or painful. The patient has had no fever, nausea or vomiting, but has not had a bowel movement for about five days and states it hurts to walk because of bilateral leg pain. His vital signs are normal. You notice that he has a number of purpuric appearing lesions on his upper extremities, thighs and buttocks. His abdomen is diffusely tender but not peritoneal.
What is your next step? What workup does this require? What would you tell the parents?
Henoch–Schonlein Purpura (HSP)
HSP is an immune-mediated small vessel vasculitis. Compared to purpura commonly caused by thrombocytopenia, the etiology of the purpuric lesions in HSP are secondary to IgA-mediated immune complex deposition in the skin. HSP typically affects children ages 2-8 years and is the most common vasculitis that is seen in this population. The rash can also have a varied appearance, usually starting as erythematous papules, with the ultimate development of palpable purpura. The diagnosis is classically pain with the triad of purpuric rash (typically on the lower limbs, buttocks), joint pain/swelling, and abdominal pain. In the majority of cases, the development of the rash typically follows a viral URI, typically about ten days.
HSP can affect multiple organ systems including the skin, kidneys, intestines as well as the body’s joints. For the skin, it involves the characteristic rash and immune complex deposition. In the kidneys, the vasculitis can cause hematuria, proteinuria and renal insufficiency. For the GI system, HSP classically can increase the risk of intussusception (likely secondary to the enlargement of the Peyer’s patches in the small intestine, leading to traction points). The intussusception is classically small bowel to small bowel intussusception in HSP, but can occur at any point in the intestinal tract.
HSP is typically self-limited, and the symptoms may last anywhere from one to four weeks. Treatment is largely supportive, and patients often require IV fluids and pain control for their symptoms. In cases with renal involvement, steroids may be beneficial.
How should I work this patient up?
Many providers will start with a urinalysis to check for hematuria and proteinuria. If these are present, then it is reasonable to obtain lab work to determine the patient’s creatinine. If the patient demonstrates abdominal pain or has a history of bloody stools, an ultrasound to rule out intussusception should be performed. The patient should be recommended routine follow-up with their primary care physician for blood pressure checks and urine samples to monitor for ongoing renal involvement/injury. Those with renal involvement, as noted by hypertension, proteinuria, or elevated BUN/creatinine, may require evaluation by a pediatric nephrologist.
Henoch-Schonlein purpura is a fairly common entity seen in pediatric emergency medicine practice. Patients present to the ED often for abdominal pain, joint/extremity pains or difficulty with ambulation, or the characteristic rash described above. Sometimes the rash can look petechial or ecchymotic as well. In patients with atypical rash features, the rash plus leg/joint pain can mimic that of new onset leukemia, so if I am at all worried, I check a CBC in these patients (and check renal function at the same time). As mentioned above, I obtain abdominal ultrasounds in those patients with abdominal pain to evaluate for intussusception. In HSP, the intussusception is classically small bowel to small bowel, however it can happen anywhere in the GI tract, hence the importance of obtaining the ultrasound. If it is small bowel-small bowel intussusception, the patient is just treated with supportive care, but if patient has an ileocolic intussusception, they would require reduction.
The most important sequela of HSP is persistent nephropathy from the immune-complex deposition (similar to IgA nephropathy). Therefore it is important to tell patients to follow up with their primary care provider, and if there is any evidence of nephropathy during my evaluation, I will refer them to nephrology at time of discharge. It is not my routine practice to prescribe steroids for HSP, unless the patient is having severe symptoms or if there is evidence of renal involvement on my evaluation. I find that talking with the nephrologist can also be helpful when deciding whether to start steroids.
Courtesy of Dr. Tomlinson
The patient was worked up regarding his abdominal pain, and his ultrasound was negative for findings consistent with intussuception. Urinalysis was negative for blood or protein. He had an abdominal xray which showed significant stool burden consistent with constipation and had symptomatic improvement after enema. His blood pressure was slightly elevated for his age range and was advised to followup with his pediatrician in the next few days for repeat blood pressure check and urinalysis.
Faculty reviewer: Dr. Tomlinson
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