DESCRIPTION OF CASE
A 23-month-old girl presented with a 24-hour history of a rash, fever, and painful soft tissue swelling of her forearms, hands, and feet. In the 2 weeks prior to this she had been suffering from symptoms of an upper respiratory tract infection and was diagnosed with tonsillitis by her general practitioner. The child had no known comorbidities and had not taken any recent medication.
Her first clinical signs (Image A: Day 0), were of urticated plaques on her arms, legs and cheeks, mimicking acute urticaria. There was associated subcutaneous oedema of her elbows, hands, and feet. By day 1 (Image A: Day 1), these lesions developed into petechiae and small purpura.
Image A.
Over the following day (Image A: Day 2), the lesions evolved into a florid purpuric rash which was concerning for meningococcaemia and the patient was therefore commenced on empirical antibiotics by the general paediatrics team. Investigations demonstrated a raised white cell count of 30 (5 to 17 × 109/L), neutrophils of 22 (1.5 to 8.5 × 109/L), C-reactive protein of 109 [0 to 5 mg/L]), normal renal function, blood pressure, and urine dipstick. Her viral serology and autoantibody screen was negative. At this point, she was seen by dermatology and a skin biopsy was performed which demonstrated a leukocytoclastic vasculitis. Direct immunofluorescence did not identify IgA deposition in dermal blood vessels.
By Day 5 (Image A: Day 5), the subcutaneous oedema had improved. However, the rash had evolved from a purpuric morphology, to a targetoid appearance mimicking erythema multiforme. The child remained systemically well throughout and her WCC and C-reactive protein rapidly normalized. She had no truncal or mucosal involvement at any point. The rash continued to fade, and had virtually resolved at 2-week follow-up without active treatment.
DIAGNOSIS AND DISCUSSION
Based on the clinical and histological features, a diagnosis of acute haemorrhagic oedema of infancy (AHOI) was made.
AHOI is a rare self-limiting small-vessel cutaneous vasculitis, perhaps representing a milder subtype of Henoch Schoenlein purpura (HSP). The condition was first described by Snow in 1913 (1), and is characterized by a triad of fever, rash, and importantly, subcutaneous oedema. The rash typically affects the face, ears, and limbs, with subcutaneous oedema affecting the distal limbs. This condition is most commonly seen in children between the ages of 4 months and 2 years (whereas HSP typically affects children aged 3 to 6 years) (2). Triggers include viral upper respiratory tract infections (most common), vaccinations, and drugs such as penicillins.
AHOI can have an alarmingly acute onset. However, its course is benign and self-limiting (3). Diagnosis is challenging, as the rash can evolve through urticarial, purpuric, and targetoid morphologies, as in our case, and therefore can mimic other entities (4). A blistering variant has also been reported (5). Although the natural evolution of AHOI has been described, this is the first report demonstrating this through serial photography. Resolution is within 1 to 3 weeks, with no long-term sequelae.
AHOI is a clinical diagnosis. Skin biopsy is not required, however can help exclude these other conditions. Differential diagnoses are broad, and depend on which stage in the evolution of the rash the patient is reviewed. These can include urticaria, urticarial vasculitis, erythema multiforme, HSP, physical abuse, and meningococcal purpura. If skin biopsy is performed, a leukocytoclastic vasculitis is seen histologically (6). As in HSP, up to a third of cases can have IgA deposition in the dermal vessels on direct immunofluorescence. Only very rarely have the systemic manifestations seen in HSP been reported in AHOI (3), however one case series found that 50% of patients had some degree of systemic involvement. This included joint involvement, gastrointestinal haemorrhage, microscopic hematuria, and compartment syndrome of the limb, although these were small numbers (7). Patients are managed with supportive care, as in our case.
In conclusion, AHOI is a self-limiting condition that can masquerade as other entities, at different points in its evolution. Clinicians must remain cognizant of the natural progression of this condition in order to avoid misdiagnosis and subjecting patients to unnecessary investigation and treatment.
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Potential Conflicts of Interest: All authors: No reported conflicts of interest. All authors have submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest. Conflicts that the editors consider relevant to the content of the manuscript have been disclosed.
References
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