Introduction:
Acute hemorrhagic edema of infancy (AHEI) is known to be a condition that is characterized by benign leukocytoclastic vasculitis. It is distinguished from other conditions by the appearance of oval, purpuric, and ecchymotic lesions on the extremities and face, petechiae, fever, and edema. It is primarily seen in infants or young children. It is also known as Finkelstein disease, Seilmayer syndrome, infantile postinfectious cockade edema, and purpura en cocarde avec edema.
What Is Acute Hemorrhagic Edema of Infancy?
The condition in which benign leukocytoclastic vasculitis can be seen is called acute hemorrhagic edema of infancy (AHEI). The primary symptoms are oval, cockade purpura, petechiae, fever, runny nose, mild cough, and swelling. Infants and young children generally suffer from it. It develops in infants aged from 4 to 5 months to toddlers of 2 years old. The cause of the condition is unknown, and this condition is idiopathic. Ecchymotic rashes are seen on the face and ears, which are distinct in appearance, and edema or swelling on the extremities. The acute hemorrhagic edema of infancy is a self-limiting condition that resolves automatically. Also, some other infectious conditions were observed in the patients with acute hemorrhagic edema of infancy (AHEI), such as escherichia coli urinary tract infection, herpes simplex gingivostomatitis, tonsillitis because of streptococcus pyogenes and respiratory tract infection by adenovirus. The condition can sometimes also be seen in association with coxsackie virus infection.
What Is the Etiology of the Acute Hemorrhagic Edema of Infancy?
It is stated that the cause of acute hemorrhagic edema in infancy is idiopathic. It is observed that the acute hemorrhagic edema of infancy is similar to the Henoch-Schoelein purpura. It is said that it can be considered a gentle version of the Henoch-Schoelein purpura, but no evidence is present to verify it. The condition is a complex immune disorder, according to many.
Several possible causes are stated below:
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Infections of streptococcal or staphylococcal origin.
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Pneumonic infections.
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Viral infections from cytomegalovirus (CMV).
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Pulmonary infections such as tuberculosis.
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Due to the use of Cephalosporins, Penicillins, and Trimethoprim-sulfamethoxazole (Trimethoprim + Sulfamethoxazole).
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Due to immunizations.
What Are the Clinical Features of Acute Hemorrhagic Edema of Infancy?
Rashes can be seen on the face, ear, and extremities. The purpura, ecchymotic lesions, and petechiae can be observed rapidly growing and spreading within the initial 12 to 48 hours. A raised, itchy rash is known as urticarial wheals, hives, or nettle rash; welts can be seen too. These rashes spread primarily across the face, ears, and extremities; the trunk area is usually spared. The lesions appear cockade or rosette-shaped. Tender edematous conditions or swellings on the upper and lower extremities are present in several cases. Localized edematous skin can be seen around the purpuric lesions. Fever can be persistent but is a low grade in the acute hemorrhagic edema of infancy. The condition reaching within the body and affecting internal organs is rarely seen, but few researchers document conditions like arthritis, nephritis, and the affected gastrointestinal tract.
How Is Acute Hemorrhagic Edema of Infancy Diagnosed?
Acute hemorrhagic edema of infancy is diagnosed by observing the clinical grounds of the disorder. The rashes are inspected and distinguished from similar conditions like urticaria and Kawasaki disease. A skin biopsy is performed if needed. Diagnosis must be made correctly by distinguishing the condition from similar conditions. The purpuric lesions are observed properly for edematous tender protrusions of the skin. The histopathological patterns of infancy's acute hemorrhagic edema mimic Henoch-Schoelein purpura's patterns, yet there is a difference in the antibody staining on direct immunofluorescence. Urinalysis may be done and is usually normal. Erythrocyte sedimentation rate (ESR) is also calculated, which either is normal or elevated in a few cases. Complete blood count and clotting time are some of the tests performed in the hematologic workup. A liver function test can also be performed to notice if there are any abnormalities. Serological studies are also carried out to differentiate acute hemorrhagic edema of infancy from other similar conditions.
What Are the Differential Diagnosis of Acute Hemorrhagic Edema of Infancy?
Many diseases, disorders, and conditions are similar to acute hemorrhagic edema of infancy, such as follows:
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Acute Febrile Neutrophilic Dermatosis: It is also known as sweet syndrome. Tender plaque and nodules can be seen along with other features such as fever, arthralgia (pain in the joints), oral lesions, genital lesions, etc. The diagnosis is made by observing the erythematous lesion’s distinct features.
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Acute Urticaria: It is also known as hives. It is a common condition that affects the population at least once in a lifetime. Allergic reactions generally cause rashes in this condition.
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Erythema Multiforme: A skin reaction caused by an infection or medicine is called erythema multiforme. Erythematous rashes can also be seen in this condition.
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Henoch-Schoelein Purpura: This disorder has many similarities with acute hemorrhagic edema of infancy. It is characterized by fever, rash on the extremities, mostly the legs, and inflammation of skin, joints, or kidneys. It is also a form of vasculitis.
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Purpura Fulminans: Microvasculature and rashes on the body can characterize it.
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Kawasaki Disease: It has clinical features such as fever, swollen legs, peeled skin, erythematous tongue, and eyes.
What Is the Treatment for Acute Hemorrhagic Edema of Infancy?
The acute hemorrhagic edema of infancy is known as a self-limiting disease and gets cured on its own in 2-3 weeks. There is no proper treatment for the cure of this condition. The use of steroids and antihistamines is not supported due to various reasons. Sometimes, corticosteroids are prescribed to pacify the acute symptoms. The patient's hospitalization is not generally required until the condition is present, along with any other serious illness; the patient may need monitoring. The condition does not affect the internal organs generally, so no specific care has to be taken, such as following a specific diet; still, a properly nutritious diet should be taken. A dermatologist should be included in the team of physicians if there is doubt regarding the diagnosis. A nephrologist and gastroenterologist can also be considered if renal involvement is seen.
Conclusion:
The acute hemorrhagic edema of infancy has male predilection, which can be found across all races and places. Patients with acute hemorrhagic edema in infancy are not toxic to others. The swelling or edema can be seen on the ears, face, and extremities. The lesions have demarcated, rosette-shaped plaques with purpura features. It gets cured automatically after 2 to 4 weeks. Recurrent occurrence of the condition can occur after it gets cured.
