Acute hemorrhagic edema of infancy: a rare case report from Syria

Introduction and Importance:

Acute hemorrhagic edema of infancy (AHEI) is a rare leukocytoclastic vasculitis clinically characterized by the classic triad (palpable purpuric skin lesions, edema, and fever) and is commonly misdiagnosed as Henoch–Schönlein purpura. AHEI frequently occurs after infections, drug treatment, or vaccination, even though its etiology is undetermined. Besides having a sudden onset, AHEI is also characterized by a self-limiting course with complete and spontaneous recovery within 1–3 weeks.

Case Presentation:

We report a rare case of a 1-year-old Syrian infant who presented to the clinic with an abnormal rash covering his entire body following a viral respiratory infection. On physical examination, he had many purpuric lesions across his body, and laboratory tests revealed that they were within the normal range. AHEI was determined based on clinical evaluation and laboratory analysis.

Discussion and Conclusion:

The authors focus on this entity as a differential diagnosis of his Henoch–Schönlein purpura. To prevent potentially serious complications, doctors should recognize purpura lesions in children exposed to respiratory infections, which received particular drugs or were given vaccinations. Furthermore, there is no danger associated with this disease, and it is benign.

Introduction

Highlights

• Acute hemorrhagic edema of infancy is a rare leukocytoclastic vasculitis clinically characterized by the classic triad (palpable purpuric skin lesions, edema, and fever) and is commonly misdiagnosed as Henoch–Schönlein purpura.

• We report a rare case of a 1-year-old boy who presented with an unusual rash due to acute hemorrhagic edema of infancy.

• It is necessary to contribute to the publication of such cases to identify more symptoms and characteristics.

Acute hemorrhagic edema of infancy (AHEI), also known as Finkelstein–Siedlmeier syndrome, is a rare skin disorder that causes leukocytoclastic vasculitis1. That usually affects children aged 2 months to 60 months and is more common in males than females2, which was first diagnosed in 19133. This syndrome has a distinct clinical triad of edema, fever, and large bruises4. Symptoms of this syndrome appear on multiple body sites, including the face, groin, and extremities5, and within 1–3 weeks after its onset, start to disappear1. Although the etiology of the disease is not clearly understood at this time, some reports in the literature suggest some potential explanations, such as the existence of an infection that occurred previously and contributed to the development of the disease in more than 75% of cases (urinary infection – upper respiratory infection – otitis media – pneumonia)6. Other contributing factors include using specific medications and antipyretics, such as paracetamol, and getting particular vaccinations, such as the triple vaccine (DPT – diphtheria, pertussis, and tetanus)7. Clinical and histological criteria are used to make a diagnosis.

Despite the terrifying appearance and severity of the lesions, the condition is benign and transient, and parents have no reason to fear it. On the other hand, systemic concerns include gastrointestinal discomfort (intussusception), arthritis, and different degrees of renal involvement have been documented8. Most recent AHEI studies have focused on disease complications; large case series are not yet available to increase our understanding of the disease’s usual presentation. We present one of the clinically rare cases in the medical literature showing a circular hemorrhagic rash. This condition has been reported in medical journals worldwide in over 300 cases9, with Syria reporting the first case.

Case presentation

A 1-year-old child was referred to the hospital with abnormal skin lesions on his body with a history of coryza, viral upper respiratory tract infection for several days, and a hemorrhagic rash that spread suddenly within 24–36 h. The mother thought it was a rash from an insect bite, but the child fell asleep and woke up with a terrible rash on her face and body, as shown in Figure 1.

Figure 1.

The appearance of a rash on the face, the upper and lower extremities, and the auricles, with pronounced edema of the hands.

He had a ring-shaped rash with hemorrhagic edema on his upper and lower extremities, cheek muscles, auricles, and to a lesser extent, his trunk. He also had swollen hands without joint pain, limitation of movement, or mucosal bleeding. In addition, swelling and bleeding were not observed in the genital area. The skin lesions were 2–3 cm in size and had diffuse margins covering most of the body surface. There was no history of vomiting, diarrhea, or food abstinence. Upon physical examination, it was discovered that the distal extremities of the upper and lower limbs and the cheekbones had purpuric confluent components with a cockade pattern, sparing the trunk and back, his vital signs were stable, and his rectal temperature was 37.4°C. Comprehensive biochemistry, coagulation testing, and a complete blood count revealed average results. A few days after admission, he was referred to a dermatologist for topical treatment using topical moisturizers and ointments to relieve his symptoms. AHEI was determined to be the cause based on clinical and laboratory results. Within a week of the onset of symptoms, the blemishes and edema gradually resolved, and the skin changed color (Fig. 2).

Figure 2.

Return of the child to its usual position with the disappearance of edema after 2 weeks.

Discussion

Acute hemorrhagic edema in infants is a rare but benign, temporary, complex immune response characterized by leukocytic microscopic polyangiitis with bruising-like skin lesions occurring in most areas of the body, followed by edema of the upper and lower extremities. The AHEI is also known as Finkelstein syndrome, postinfectious iris purpura, edema, medallion purpura, or Seidlmayer syndrome. Snow first described this condition in 1913. The child’s state is stable, and the disease is recognized and diagnosed by her three characteristic symptoms: fever, purpura, and edema. A skin biopsy can help confirm the disease.

Laboratory tests are often normal (including complete blood count, clotting time, urine, liver, and kidney tests)10. Therefore, clinical examination is not qualitative, but diagnosis of the disease mainly depends on physical examination. There is no definite cause leading to the development of the disease, but some drugs, vaccinations, and infectious diseases are thought to be factors that influence it. Cytomegalovirus, herpes simplex virus, tuberculosis, Streptococci, Staphylococci, and pneumonia are closely associated with this rare disease11.

This AHEI is considered a self-limiting disease. The child will recover on its own within a few weeks. The symptoms of this condition can be managed by giving patients systemic corticosteroids, even though these drugs are unnecessary and recovery remains feasible without them. Currently, there is no effective treatment for this condition12. Recurrences are not uncommon13. In our case, the history began with bruises on the face and ears, then extended to the feet, hands, lesser extent, and the trunk14. One of the most important differential diagnoses for this disease is Henoch–Schönlein purpura, which may pose a problem for physicians due to their similar histopathological structure. After being given a skin ointment to relieve the rash and edema, the child recovered within a week, and no new lesions appeared.

This work has been reported in line with the SCARE (Surgical CAse REport) 2020 criteria15.

Conclusion

AHEI is a self-limiting, benign condition that is rare in most cases. Doctors should notice purpura lesions in children exposed to respiratory illnesses, be given specific medicines, or be vaccinated to avoid potentially severe problems.

Ethical approval

Ethical approval was also taken from the Faculty of Medicine at Al-Baath University.

Patient consent

Written informed consent was obtained from the patient’s parents, who have a 1-year-old child, for the publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.

Sources of funding

None.

Author contribution

J.A. and H.H.: writing the manuscript; J.A.: drafting the article; J.A. and E.A.: critical revision of the article for important intellectual content.

Conflicts of interest disclosure

The authors declare that they have no conflicts of interest.

Research registration unique identifying number (UIN)

1. None.

Guarantor

Jamal Ataya, MS in Medicine, University of Aleppo, Syria. E-mail: dr.jamalataya@gmail.com.

Provenance and peer review

Not commissioned, externally peer-reviewed.