Kaposi's Varicelliform Eruption: A Serious and Potentially Fatal Complication of Darier's Disease

Darier's disease (DD) is an autosomal dominant genetic disorder characterized by skin barrier dysfunction and chronic skin inflammation predisposing patients to secondary infections. Kaposi's varicelliform eruption (KVE), a severe viral infection, most commonly caused by herpes simplex virus (HSV), represents a life-threatening complication in these patients. The authors present the case of a 48-year-old woman with DD, who developed a progressive painful vesiculopustular eruption accompanied by systemic symptoms, including fever, bilateral otalgia, and ocular pain. The initial skin lesions, confined to the scapular region, rapidly spread to the face, periorbital region, and external ears. Clinical examination revealed vesiculopustular lesions, conjunctival hyperemia, and ear involvement. Laboratory testing confirmed HSV-1/2 immunoglobulin M (IgM) positivity, and blood cultures grew Staphylococcus aureus, indicating bacterial superinfection. The patient was treated with intravenous acyclovir and flucloxacillin, leading to significant clinical improvement. Clinicians should maintain a high index of suspicion for KVE in patients with DD presenting with vesicular rashes, particularly when systemic symptoms or rapid progression is observed. This case underscores the importance of early recognition of KVE in DD patients to facilitate timely antiviral and antibacterial treatment, reducing the risk of severe complications such as vision loss and permanent auditory impairment.