Acute Hemorrhagic Edema of Infancy.

Sir,

Acute hemorrhagic edema of infancy (AHEI) was originally described by Snow in the USA in 1913.[1] Finkelstein gave a detailed description in Europe in 1938 and since then it has been recognized in the European literature under various terms: Finkelstein disease,[2] Seidlmayer syndrome,[3] infantile postinfectious iris-like purpura and edema, purpura en cocarde avec edema.[4] AHEI is a benign, small vessel vasculitis of young children with characteristic skin findings. The cutaneous findings are dramatic, both in appearance and rapidity of onset; histopathology is characterized by leukocytoclastic vasculitis (LCV).[5]

An 8-month-old otherwise healthy infant was referred from the pediatric department in view of skin lesions on various body parts. The baby had multiple red angry looking raised lesions over the face, both upper and lower limbs and buttocks. The parents informed that the baby had a mild cough and cold since 3 days for which he was receiving syrup amoxicillin-clavulanate and syrup paracetamol (15 mg/kg). On examination, the child was active, playful and nontoxic with stable vitals except mild fever, facial puffiness, and pedal edema. Cutaneous examination showed multiple oval to round ecchymotic patches and plaques over the face, arms, hands, gluteal area, legs and feet varying in size from 2 to 5 cm in diameter [Figure 1]. Systemic examination was normal for age. Routine laboratory workup, including urinalysis was within normal reference limits. Biopsy and immunofluorescence study was deferred as parents did not give consent for the same. With a clinical diagnosis of AHEI, we treated the baby symptomatically and prescribed topical application of calamine lotion with subsequent follow-up after 3 days. On second follow-up visit, the original lesions showed resolution with pigmentary changes but revealed the appearance of new lesions at the adjoining areas. The baby was additionally prescribed syrup cetirizine and was asked to follow after 3 more days. Third follow-up showed complete resolution of skin lesions with mild pigmentary changes [Figure 2].

Figure 1

Multiple ecchymotic patches and plaques on buttocks, legs and face associated with facial puffiness

Figure 2

Complete resolution of lesions after 1-week symptomatic management

Acute hemorrhagic edema of infancy is an acute LCV occurring in infants and young children between the ages of 3 months and 2 years. Since its first description in 1913, approximately 100 cases of AHEI have been reported.[6] AHEI is an immune complex-mediated vasculitis, which is probably initiated from prior bacterial or viral infections (mostly upper respiratory tract and urinary infection) in addition to some medications especially antibiotics and with less probability, vaccination.[7] The peak incidence is during winters when there are high chances of respiratory infections in the target population.[8] Males are more susceptible than females.[9] In contrast to striking cutaneous lesions and rapid progression, the overall general condition is good and babies are nontoxic.[10] Painless non-pitting edema of the face, mostly asymmetric, may be the first clinical sign of AHEI. Later on, painful ecchymotic patches and plaques appear on the face and extremities or large target shaped lesions may erupt suddenly. Rarely, scrotum may be involved in male infants. Skin biopsy shows features of LCV with fibrinoid necrosis and neutrophilic infiltration of the vessel wall. The differential diagnosis of AHEI includes Henoch–Schönlein purpura (HSP), meningococcemia, and erythema multiforme and drug eruption. The chief differential diagnosis of AHEI is HSP. The age of onset of AHEI (2–24 months) is lower when compared to HSP (4–7 years). Systemic complications (arthralgia, gastrointestinal bleeding, and nephritis) are common in HSP.[11] HSP can distinguished clinically by the presence of palpable purpura on the extensor surface of legs and buttock whereas, in AHEI, face and extremities are prominently involved and is accompanied by severe widespread edema.[12] Histopathological findings in AHEI are very similar to HSP. However, IgA deposition is seen in only 10–35% cases of AHEI.

There is no effective treatment for AHEI and symptomatic care has been advocated. The temptation to prescribe systemic steroid should be resisted as the condition is self-resolving with excellent prognosis and no sequelae. Spontaneous recovery usually occurs within 1–3 weeks without any scars. Relapse of the disease is also reported.[13]