Acute haemorrhagic oedema of infancy

Description

We present a previously healthy 9-month-old girl which presented to our emergency department with coryzal-like symptoms, a 3-day history of fever and rigours and large purpuric plaques with irregular, annular and targetoid shapes, located on the face, hands and perineum and sparing the trunk, which had appeared in the previous 24 h and were still progressing (figures 1 and 2). There was bilateral oedema of the hands and feet. She had stopped amoxicilin 4 days earlier for acute otitis media and the recent vaccination history was not stated. Upon examination, she appeared well and she was normotensive. Her urinalysis was normal as were her full blood count and clotting screening. The C reactive protein was 5.3 mg/l and her blood culture was negative. A dermatologist's consultation confirmed the diagnosis of acute haemorrhagic oedema of infancy (AHEI).

Figure 1.

Large purpuric plaques with irregular and annular shapes located in the face.

Figure 2.

Purpuric plaques with irregular and targetoid shapes in the hand.

The child received only symptomatic treatment and there was regression of the lesions in 2 weeks.

AHEI is a leucocytoclastic vasculitis, involving the small blood vessels in the dermis. It typically occurs in children between the ages of 4 months–2 years and is self-limited. Its aetiology remains unknown, although some consider AHEI an immune complex-mediated disease.^{1 2}

The classical features of AHEI are fever, oedema and rosette-shaped, annular-shaped or targetoid-shaped purpura affecting the face, ears and extremities, in a well-appearing child. The onset is sudden and the oedema may be painful. Systemic symptoms such as abdominal pain, gastrointestinal bleeding, arthritis and nephritis are rarely reported.²

The differential diagnosis of AHEI includes Hennoch-Scönlein purpura, menigoccocemia, hypersensitivity vasculitis, Kawasaki disease, drug eruption, urticaria, malignancies and child abuse. Histological findings usually confirm the diagnosis. Treatment is symptomatic.^{2 3}

Learning points.

• Although haemorrhagic oedema of infancy (AHEI) has an alarmingly acute onset, the course is benign and self-limiting.

• The differential diagnosis of AHEI includes meningococcemia; however, patients with meningococcaemia usually are sick-looking.

• Early recognition by physicians should prevent confusion with other more severe conditions.