Morning Report

Early this fall, an 11-month-old baby boy developed a mild cough without fever, which persisted for four days. He was brought to the Fast Track area of the Emergency Department at Nemours/Alfred I. duPont Hospital for Children where he was treated with albuterol for wheezing. He was discharged to home and went to see his primary care physician the next day for a recheck. At that time, the baby had mild wheezing. No rash was present and he was given amoxicillin. The next day he was noted to have an unusual rash on his lower extremities and his parents brought him back to our Emergency Department.

In the ED, it was noted the baby had rhinorrhea, no vomiting or diarrhea. He had been drinking well, sleeping a bit more than usual, but there were no other concerns. The baby was full term and had an unremarkable medical history. His immunizations were up to date and his only medications included amoxicillin, albuterol and acetaminophen. No other family members were ill at home.

The baby appeared well in the ED. Vital signs were T-37.6 C, P-134/min, RR-32/min, BP 98/60. He was active and in no distress. His head was normal, AFOF. His pharynx was clear, there were no oral lesions, and the pediatric resident noted moist mucous membranes. His neck was supple and his chest exam, including cardiac exam was unremarkable. His abdomen was soft, not tender and there was no mass or organomegaly. His skin exam revealed multiple purpuric lesions on the face and all extremities. There was no rash on the chest, back or abdomen. There were petechiae on the right forearm after a tourniquet was placed and blood was drawn. The extremities appeared edematous but there was no apparent pain when the baby moved any joints. The baby had normal genitalia with no swelling of the scrotum.

Laboratory studies revealed WBC 12,300, hemoglobin 11.9, hematocrit 33, and a platelet count of 412,000. A chemistry panel showed: Na 141, K 5.0, Cl 103, TCO2 20, Glucose 200, BUN 12, Creat 0.4, glucose 92 and calcium 10.6. Urinalysis had a specific gravity of 1.030, negative for glucose, protein, nitrites, leukocyte esterase. Trace blood was present.

The perceptive senior resident who presented the case at Morning Report focused on the purpura and its location on the face and extremities. She knew that a purpuric rash that spared the trunk was suspicious for a condition such as Henoch Schonlein Purpura (HSP). The normal platelet count was reassuring and also pointed to a vasculitis such as HSP. The resident was somewhat concerned about the infant’s age and wondered if HSP could occur in such a young baby. Infection was considered, but the baby had no fever and the purpuric rash was only present in distinct areas. Drug reaction was also considered since the baby had taken a few doses of amoxicillin just prior to the onset of the rash. But again, since the rash was only in limited areas, this seemed less likely.

Fortunately, distinguished faculty members from our Division of Rheumatology were present at Morning Report. Carlos Rose, MD and AnneMarie Brescia, MD were intrigued by the infant’s presentation and commented on this interesting case, agreeing that the presentation was consistent with a vasculitis. The likely diagnosis was Acute Hemorrhagic Edema of Infancy. This unusual condition, an acute cutaneous leukocytoclastic vasculitis, resembles HSP. Acute Hemorrhagic Edema of Infancy affects children younger than 2 years of age. They typically present with mild fever, hemorrhagic skin lesions and edema of the face and extremities. There are no specific laboratory findings, although thrombocytosis is common, as noted in our patient. Infants with this condition may also have leukocytosis and a mildly elevated sedimentation rate. Circulating immune complexes can be detected.

The good news is, infants with Acute Hemorrhagic Edema of Infancy have a short, benign course, with the rash generally lasting 1-3 weeks. GI bleeding and renal complications are very rare. Proteinuria and hematuria are even less likely than with HSP. The etiology for Acute Hemorrhagic Edema of Infancy is unknown. Although the condition mimics HSP, it may be a separate entity. Immunizations, medications and infections, such as upper respiratory infections, are sometimes considered as the cause. A second episode of the condition rarely occurs.

This baby was discharged from AIDHC at day’s end. Although he made for an interesting discussion at Morning Report, hospital admission (in retrospect) was probably not necessary. Steroids and other anti-inflammatory agents are usually not needed, as the condition resolves spontaneously. This infant was managed with meticulous supportive care at home. Close follow-up revealed he did quite well. His rash resolved over the next 10 days.

Reference: Karremann M, Jordan A, Bell N, et al. Acute Hemorrhagic Edema of Infancy: report of four cases and review of current literature. Clinical Pediatrics 2009; 48:323.

Steven M. Selbst, MD