Acute Hemorrhagic Edema of Infancy: an unusual diagnosis for the general pediatrician

Abstract

Acute Hemorrhagic Edema of Infancy (AHEI) is a rare leukocytoclastic vasculitis, clinically characterized by the classical triad: palpable purpuric skin lesions, edema and fever, and is commonly misdiagnosed as Henoch-Schönlein purpura. In addition to its sudden onset, AHEI is also characterized by its self-limited course with complete and spontaneous recovery occurring between 1 and 3 weeks. Because of the scarcity of studies on therapy with corticosteroids, the conservative approach is usually recommended. The authors report an unusual case of an one-year-old boy who presented with typical cutaneous rash of AHEI and orchitis, the latter showing complete resolution after less than 24 hours of prednisolone therapy. The authors call attention to this entity mainly as a differential diagnosis of Henoch-Schönlein purpura and to the importance of new studies to establish the benefits of corticosteroid therapy for AHEI.

Acute Hemorrhagic Edema of Infancy after Coronavirus Infection with Recurrent Rash

“…Several case reports note rapid improvement of purpura or edema after the administration of corticosteroids [ 3 , 11 ]. Given the improvement in our patient's purpura by 48 hours after onset, we made the decision not to administer corticosteroids.…”

“…This suggests that some previously reported patients may have improved even without corticosteroids, though at least one case has noted relapse of symptoms when steroids were removed [ 7 ]. While use of corticosteroids remains controversial, most reports suggest that corticosteroids only be considered in severe presentations with complications or inability to maintain fluid intake [ 7 , 11 , 12 ].…”

Coronavirus Disease 2019 and Acute Hemorrhagic Edema of Infancy

“…Usually, AHEI is a self-limited disease, and more than 90% of infected patients completely recover from the disease only with a supportive care within 1–3 weeks after the symptoms' presentation. Of note, systemic involvement, including abdominal pain, intussusception, arthralgia, testicular torsion, and glomerulonephritis, occurred in fewer than 10% of patients diagnosed with AHEI [ 4 , 5 ].…”

Acute hemorrhagic edema of infancy (AHEI): Alarming cutaneous presentation of a benign and self‐limited disease

“…AHEI, also known as Finkelstein or Seidlmayer disease, is a rare cutaneous leukocytoclastic small vessel vasculitis, which mainly affects male infants and young children aged 6–24 months. 4 , 5 The etiology is unclear, although viral and bacterial infections (cytomegalovirus, herpes simplex virus, varicella zoster virus, adenovirus, rotavirus, streptococci, staphylococci, and tuberculosis) of respiratory, urinary, and gastrointestinal tracts may be observed in some affected patients. 6 , 7 , 8 , 9 , 10 Vaccinations and medications (penicillin, cephalosporin, trimethoprim–sulfamethoxazole, paracetamol, thiazides, and nonsteroidal anti‐inflammatory drugs) may also be involved in its pathogenesis.…”