Acute generalized exanthematous pustulosis (AGEP) is an abrupt cutaneous adverse reaction usually in response to medications. It is generally a self-limiting disease if diagnosed promptly and the offending agent discontinued. Cetirizine, a commonly used anti-histamine medication for the treatment of allergic diseases has few reported side effects and is normally well-tolerated and effective. Herein, the first reported case of cetirizine induced AGEP is presented, followed by a discussion of the clinical and pathological aspects of this adverse cutaneous reaction to a widely used drug. Awareness of this reaction is vital owing to the extensive use of cetirizine and the importance of drug cessation once the reaction is identified. Lastly, other pustular cutaneous reactions may present similarly and therefore accurate identification of this disease can prevent unnecessary diagnostic testing.

Background and purpose

PHACE is an acronym for posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities. Several case reports of arterial ischemic stroke (AIS) in individuals with PHACE have been published, but risk factors for AIS in PHACE have not been clearly defined. The objective of this article is to review all cases of stroke in PHACE in children and describe clinical characteristics that may be associated with an increased risk of AIS.

Methods

A literature and registry search was conducted to identify patients with PHACE who had experienced AIS. Data were analyzed to determine age of onset, presenting signs and symptoms, and clinical features among this cohort compared with PHACE without AIS.

Results

Twenty-two individuals with PHACE and AIS were identified. Imaging of the arteries of the head and neck was reported in 20 of 22. Narrowing or nonvisualization of at least 1 great cerebral vessel was present in 19 of 20 and of those, 15 had ≥ 2 vessels involved. Aortic arch anomalies were reported in 13 of 22 individuals.

Conclusions

Aplasia, hypoplasia, or occlusion of a major cerebral artery appears to be a significant risk factor for AIS in children with PHACE, especially when >1 vessel is involved or if there is coarctation of the aorta.