In midsummer, an 8-year-old girl, accompanied by her parents, presented to her pediatrician’s office for evaluation of acute right-lower-quadrant abdominal pain with nausea and vomiting. The pediatrician suspected acute appendicitis.
The girl’s white blood cell (WBC) count was somewhat elevated at 14,000/mm3. But a computed tomography scan and pelvic ultrasound revealed only a small amount of mesenteric adenitis and a normal-appearing appendix, and the pediatrician sent her home.
The following day, the patient returned to the pediatrician with continued abdominal pain and an inability to keep anything down. The results of a rapid strep test were positive, and the patient was treated with intramuscular penicillin G (Bicillin L-A) and was encouraged to increase her fluid intake and sent home.
Two days later, her parents brought the patient to our emergency department for increasing abdominal pain with persistent nausea and vomiting. The girl’s only significant past medical history was several prior episodes of strep pharyngitis. She had no known drug or food allergies.
While being evaluated in the ED, the patient appeared ill but nontoxic. The patient’s vital signs were as follows: blood pressure 113/84 mm Hg; heart rate tachycardic, from 110 to 148 beats per minute; respiratory rate 24 breaths per minute; and temperature 98.2 F (36.8 C). Her WBC count had risen from 14,000/mm3 to 18,000/mm3 with 77% segmented neutrophils, despite the administration of intramuscular antibiotics 2 days earlier. Repeat pelvic ultrasound showed free fluid located anterior and superior to the bladder (Figure 1), which had not been seen on the prior examination.
The appendix could not be visualized. The patient’s urine output was decreasing at the time of examination due to poor oral intake. On physical examination, the patient’s abdomen was mildly distended with normal tympany and dullness to percussion. Palpation of the abdomen revealed tenderness that was greatest along the umbilical and periumbilical regions and the right-lower quadrant, with mild generalized guarding and no rebound tenderness. The patient exhibited positive peritoneal signs. The remainder of the examination was unremarkable.
Surgical & Hospital Course
After extensive consultation with the patient, her family, her pediatrician and a surgeon, we decided that surgical intervention would be in the patient’s best interest, considering her worsening clinical symptoms. The patient was prepped for a diagnostic laparoscopy with laparoscopic appendectomy.
Figure1. Pelvic ultrasound showing free fluid (FF) located anterior and superior to the bladder (BL). (Click image to view larger photo.)
Figure 2. A small amount of thin, blood-tinged fluid was evident during laparoscopy, and enlarged lymph nodes were visible in the small bowel mesentery (arrows), especially in the distal ileum. (Click image to view larger photo.)
Figure 3. Figure 3. Typical presentation of purpura on the feet of a patient with HSP. (Click image to view larger photo.)
Immediately evident upon entering the abdomen was a small amount of thin, clearly blood-tinged fluid in the pelvis. The small bowel mesentery contained significantly enlarged lymph nodes, especially in the area of the distal ileum (Figure 2).
The appendix was mildly injected and questionably enlarged, so the surgeon decided to remove it. The patient tolerated the procedure well with no complications. She had an uneventful hospital recovery. Her postoperative pain, while present, was well controlled with narcotic pain medication. She tolerated an advancing diet with no episodes of nausea, and she ambulated well within a few hours after the surgery. The patient was discharged the following day on oral acetaminophen with codeine and with instructions to follow up with her pediatrician in 2 to 3 days and with her surgeon in 7 to 10 days.
Diagnostic examination of the appendix specimen revealed benign follicular lymphoid hyperplasia. No acute appendicitis was identified.
A Return to the ED
On postoperative day 2, the patient returned to the ED with a new complaint of swelling and tightness in her hands and feet. She stated that the swelling had surfaced that morning but was not painful. She reported no chest pain, shortness of breath, headache or changes in vision, nor a history of similar episodes in the past. On physical exam, obvious swelling was noted in the fingers, toes and parts of the feet. Also noted was raised urticaria over the anterior surface of both ankles. No other rash or lesions were noted.
The ED team administered diphenhydramine, epinephrine and prednisolone, then observed for 1 hour. Within 10 minutes, the swelling and urticaria of the patient’s hands and feet had resolved. She was released from the ED with instructions to continue the oral diphenhydramine for 24 hours and return if symptoms resurfaced.
Within 2 hours, the patient returned to the ED. She had developed petechiae over both feet, similar to the presentation shown in Figure 3. Rash was not evident anywhere else on her body. The patient was active, playful and nontoxic appearing. Her vital signs were stable, and she was afebrile. All laboratory values were within normal limits.
A consult was obtained from a specialist at the local university children’s hospital, who suggested the diagnosis of Henoch-Sch”nlein purpura (HSP). At the time, differential diagnoses of vasodilatory effect of epinephrine or allergic reaction to codeine also were considered. After discharge, the patient’s pediatrician placed her on 10 mg of oral cetirizine once daily. The patient’s codeine-containing postoperative pain medication was discontinued, and the patient was asked to use children’s-strength oral acetaminophen instead.
During the rest of her recovery course, the patient was closely observed for additional or worsening manifestations of HSP. Ten days after her discharge, the patient began complaining of generalized joint pain. This symptom resolved within a few days of recommended use of cetirizine and children’s-strength over-the-counter analgesics.
This case report highlights an unusual presentation of HSP, a small-vessel vasculitis disorder most prevalent in children between 3 and 10 years old. A triad of nonthrombocytopenic purpura, colicky abdominal pain or nephritis and arthritis characterizes this syndrome.1 Variability in presentation, however, makes the diagnosis difficult, and our case was unusual in that the progressive abdominal pain and gastrointestinal symptoms preceded the typical purpuric rash by several days, initially compelling the presumption of an acute abdomen.
HSP occurs twice as often in boys than in girls. It has an annual incidence of 14 cases per 100,000 people and occurs most frequently in the spring and fall,2 but not always – our patient’s symptoms occurred in midsummer.
HSP is an acute self-limiting illness, with about one-third of affected patients having one or more recurrences. Left untreated, abdominal pain resolves spontaneously within 72 hours in the majority of cases.3
Click to view larger graphic.
Etiology & Presentation
The etiology of HSP is unknown, although extensive studies have linked HSP with various environmental triggers, such as a preceding upper respiratory illness (URI) or possible drug exposures (Table 1). The research into these possible HSP triggers is not complete, and few studies have compared HSP patients with an incidence of infection or exposure with a control group.4
Nevertheless, one-half to two-thirds of children report a URI preceding the clinical onset of HSP by 1 to 3 weeks.3 Our patient was exposed to a prior URI as evidenced by the positive strep test, and she had received penicillin prior to the onset of HSP symptoms.
It is generally accepted that immunoglobulin A (IgA) plays a fundamental role in the pathogenesis of HSP.4 The correlation between HSP and IgA is indicated by elevated serum IgA concentrations and IgA deposition in vessel walls.4 This deposition in the small vessels throughout the body leads to the hallmark symptoms of HSP. IgA depositions accumulating in the small vessels of the intestinal mesentery result in HSP’s gastrointestinal manifestations (Table 2).
Numerous studies have cited GI complaints as the first symptom in patients with HSP. In one case study in South Korea,5 a 4-year-old boy presented with periumbilical and lower abdominal pain and leukocytosis. After 5 days, the patient developed joint pain, followed by the appearance of purpura several days later.
In a study conducted by Chen and colleagues in Taiwan,6 41 of 208 patients diagnosed with HSP experienced GI symptoms before the manifestation of skin lesions. Five of those patients underwent a laparotomy; four were operated on based on a suspicion of acute appendicitis or peritonitis.
Even more unusual was a case report of a young boy with HSP whose abdominal symptoms predated the appearance of purpura by 3 months.7
Due to HSP’s benign self-limiting nature, treatment is supportive. Recommendations include analgesia for pain relief, hydration and occasional use of corticosteroids for GI and inflammatory processes.
Patient and family reassurance is essential due to the condition’s unpredictable lapsing and remitting course. Additionally, vigilance is needed for signs of new or worsening symptoms, such as renal involvement and advanced GI complications.8
Appendicitis? Consider HSP
The difficulty of this case revolved around the uncharacteristic order in which the patient’s symptoms presented. When approached chronologically, our patient’s symptoms strongly suggested acute abdomen, and this is where the inherent difficulty in diagnosing HSP poses the greatest risk to patients.
When gastrointestinal symptoms present well before the telltale palpable purpura, patients might undergo otherwise avoidable surgical procedures. Putting HSP on the list of differential diagnoses for persistent abdominal pain, nausea, vomiting and leukocytosis, especially in school-aged children, might decrease the likelihood of those unnecessary surgical procedures.
1. Shetty AK, et al. Infantile Henoch-Sch”nlein purpura. Arch Fam Med. 2000;9(6):553-556.
2. Kraft DM, et al. Henoch-Sch”nlein purpura: a review. Am Fam Physician. 1998;58(2):405-408,411.
3. Scheinfeld NS, Jones EL. Pediatric Henoch-Schonlein purpura. Medscape Reference. http://www.emedicine.com/ped/topic3020.htm. Updated Nov. 23, 2010. Accessed July 25, 2011.
4. Saulsbury FT. Epidemiology of Henoch-Sch”nlein purpura. Cleve Clin J Med. 2002;69(suppl 2):SII87-SII89.
5. Park SH, et al. Gastrointestinal manifestations of Henoch-Sch”nlein purpura. J Korean Med Sci. 1990;5(2):101-104.
6. Chen SY, Kong MS. Gastrointestinal manifestations and complications of Henoch-Sch”nlein purpura. Chang Gung Med J. 2004;27(3):175-181.
7. Byrn JR, et al. Unusual manifestations of Henoch-Sch”nlein syndrome. Am J Dis Child. 1976;130(12):1335-1337.
8. Kon‚-Paut I. Review for the generalist: Henoch-Sch”nlein purpura in children. Pediatr Rheumatol Online J. 2005;3(1):43-56. http://www.pedrheumonlinejournal.org/jan-feb05/Henoch_Schoelein.htm. Accessed July 25, 2011.
Dina DeMarco is a physician assistant at Intermountain American Fork Surgical Associates in American Fork, UT. She has completed a disclosure statement and reports no relationships related to this article.