A 5-month-old male came to the emergency room with a 2 day history of a fever to 102.1° and a new onset of rash. He was a full-term infant who had been otherwise well with no medication or drug exposure or recent travel. He was not drinking well because of the fever. The family history was negative for any dermatological problems. The pertinent physical exam showed a tired male with normal vital signs. His examination was negative except for 1-3 cm purpura on the legs, buttocks, and cheeks. They were not palpable. His feet, eyes and ears were edematous. He had no mucosal involvement and only some shoddy anterior cervical and groin nodes.
The diagnosis of mild dehydration and possible Henoch-Schonlein purpura was made and the patient was admitted for further evaluation. The work-up was negative with a normal complete blood count, liver function tests, BUN, creatinine, erythrocyte sedimentation rate, C-reactive protein, complement, anti-nuclear antibodies, antistreptolysin O and numerous serologies including herpes virus, adenovirus, Mycoplasma and Rickettsia rickettsii. The patient’s clinical course showed the child developing new lesions over the next day and mildly painful edema. A dermatologist was consulted and made the diagnosis of acute hemorrhagic edema of infancy. The dermatologist didn’t recommend any specific treatment and the patient was sent home with followup after he was afebrile and drinking well. The resident who initially saw the patient also saw his brother in the emergency room later in the month and the mother said that he was well and the lesions were almost faded away.
The differential diagnosis for acute hemorrhagic edema of infancy (AHEI) is similar to purpura and includes:
- Henoch-Schonlein purpura (HSP)
- Drug induced
- Kawasaki disease
- Rocky Mountain Spotted Fever
- Trauma induced
- Infectious Disease
- Erythema multiforme
- Gianotti Crosti
- Hemorrhagic urticaria
- Sweet’s syndrome
- Child maltreatment
- Neonatal lupus
Acute hemorrhagic edema of infancy (AHEI, also called Seidlmayer or postinfectious cockade purpura, medallion-like purpura, or Finkelstein’s disease) is an uncommon, self-limited cutaneous leukocytoclastic vasculitis that usually affects children 4-24 months. Despite their appearance, the children generally feel well. It usually presents with fever, and 1-5 cm rosette- or cockade-shaped hemorrhagic purpuric lesions especially of the lower extremities, buttocks, ear, eye and face. Edema, often painful, of these same areas occurs. The skin changes have rapid onset and the coloring may evolve from red/purple to brown/yellow as the blood in the lesions is degraded. The lesions may spread to other body areas such as the upper trunk but this occurs later in the disease course. Mucous membranes are spared and visceral involvement is rare. Laboratory evaluation is basically negative including erythrocyte sedimentation rate, C-reactive protein, urine, stool, ASO titre, antinuclear antibodies, rheumatoid factor and infectious disease’s serologies. Mild leukocytosis with increased neutrophils, lymphocytes or eosinophils has been noted. Generally complements are normal but a few cases have noted transient low complement levels. Skin biopsy shows small dermal vessel vasculitis with all immunoglobulin classes being deposited. Recovery occurs in a few days or weeks, and recurrence is almost unheard of.
AHEI is felt to be an autoimmune phenomena but the actual cause is unknown. It has been associated with prodromes of mild or systemic infectious diseases (i.e. upper respiratory tract infection, otitis media, urinary tract infections, and pneumonia) medications and vaccines. Recently one case report showed positive cytomegalovirus titres.
Steroids and antihistamines have been used with patients but are not effective. AHEI was felt at one time to be a variation of HSP, but they are now considered to be distinct from each other. HSP is the major entity that is confused with AHEI. HSP usually is seen in older children (> 2 years), they usually appear unwell, visceral involvement is common, and the disease course can wax/wane for weeks and recurs 50% of the time. HSP also usually has palpable purpura of the buttocks and lower extremities and usually spares the face. IgA is the predominant immunoglobulin deposited in a skin biopsy. For more information about HSP, see Is it Really Henoch-Schonlein Purpura?
Questions for Further Discussion
1. List the most important history and physical examination findings for distinguishing a purpural rash needing immediate treatment from one that does not.
2. List other hypersensitivity reactions in infants and neonates.
- Disease: Acute Hemorrhagic Edema of Infancy | Vasculitis
- Symptom/Presentation: Edema | Fever and Fever of Unknown Origin | Rash
- Specialty: Dermatology | Emergency Medicine | General Pediatrics
To Learn More
To view pediatric review articles on this topic from the past year check PubMed.
Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.
Information prescriptions for patients can be found at MedlinePlus for this topic: Vasculitis
To view current news articles on this topic check Google News.
To view images related to this topic check Google Images.
Carder KR. Hypersensitivity reactions in neonates and infants. Dermatol Ther. 2005 Mar-Apr;18(2):160-75.
Savino F, Lupica MM, Tarasco V, Locatelli E, Viola S, Cordero di Montezemolo L, Coppo P. Acute Hemorrhagic Edema of Infancy: A Troubling Cutaneous Presentation with a Self-Limiting Course. Pediatr Dermatol. 2012 Nov 21. epub ahead of publication.
ACGME Competencies Highlighted by Case
1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
2. Essential and accurate information about the patients’ is gathered.
3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
4. Patient management plans are developed and carried out.
7. All medical and invasive procedures considered essential for the area of practice are competently performed.
8. Health care services aimed at preventing health problems or maintaining health are provided.
9. Patient-focused care is provided by working with health care professionals, including those from other disciplines.
10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.
24. Cost-effective health care and resource allocation that does not compromise quality of care is practiced.
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa Children’s Hospital