Acute Hemorrhagic Edema of Infancy
For 2 days, an 11-month-old girl had a progressively worsening rash and subjective fever (A). The rash began on the legs as bumps, which later became large violaceous lesions (B) and spread to the face, arms, and trunk. During the 12 hours before presentation, erythematous swelling of the periorbital area and ears developed. The upper eyelid swelling was the mother’s greatest concern because it limited the baby’s ability to open her eyes. The infant had had no difficulty in breathing and no emesis, diarrhea, or changes in urinary output. She had been eating less but drinking well.
The patient was alert and crying but consolable. Vital signs were normal. Oxygen saturation was 100% on room air. Weight and height were appropriate for age. Pulmonary, cardiovascular, GI, and neurological findings were normal. Multiple palpable, purpuric lesions were noted on the face, ears, trunk, and upper and lower extremities. The facial lesions were not warm, tender, or blanching. There was significant edema of the upper eyelids, pinnae, and ear lobes with dark ecchymosis. Intraoral findings were normal.
Laboratory results, including erythrocyte sedimentation rate, C-reactive protein level, coagulation factors, chemistry panel with liver enzyme levels, urinalysis, complete blood cell count, and platelet count, were all normal. Stool was guaiacnegative. Results of a rapid streptococcal screen were positive.
The infant was given 1 dose of intravenous methylprednisolone for suspected Henoch Schönlein purpura (HSP) and 1 dose of oral diphenhydramine hydrochloride because of the possibility of abdominal or joint pain, which may have been present but which the infant was unable to communicate. Mild improvement of the eyelid swelling was noted the next day (C).
Acute hemorrhagic edema of infancy (AHEI), also known as Finkelstein or Seidlmayer disease, was diagnosed. Although some experts feel that this condition is a variant of HSP, others
believe that it is a separate disease. If AHEI is a variant of HSP, one would expect that it would be similarly mediated by immune complexes. However, IgA deposition on biopsies of skin lesions is less common in AHEI than in HSP. Also, unlike HSP, AHEI typically occurs in children aged 4 to 24 months. As in HSP, presentation is often dramatic—as was the case in this infant. Often, patients have a recent history of an upper respiratory tract infection or ear infection.
AHEI is a cutaneous vasculitis that usually presents with palpable purpura or petechiae primarily on the face, ears, and extremities, similar to HSP. The first lesions appear as small macules or papules, which the family often confuses with bug bites. Over the next few days, the lesions enlarge. Edema of the face and lower and upper extremities may also develop. The arthritis/arthralgia, nephritis, abdominal pain, and GI bleeding that accompany HSP are rare in AHEI. Although routinely benign, AHEI needs to be distinguished from more serious conditions, such as meningococcemia, Rocky Mountain spotted fever, erythema multiforme, child abuse, Kawasaki disease, and septicemia.
AHEI usually resolves spontaneously within 1 to 3 weeks. The most worrisome complication is necrosis of the structures of the outer ear, especially the ear lobe, which occasionally develops in the areas of severe swelling and purpura. Treatment is symptomatic. The use of corticosteroids is controversial and not universally recommended. Although it rarely happens, the disease can recur.
This little girl presented with a classic case of AHEI. She appeared nontoxic, despite the impressive swelling of her face and ears with the purpuric rash. She recovered quickly with almost complete resolution of the rash about 4 days after presentation (D).