Eruptive pseudoangiomatosis in adults: a community outbreak

Eruptive pseudoangiomatosis (EPA) is a rare cutaneous disorder of unknown cause originally described in children1 and characterized by an eruption of distinctive erythematous angiomalike papules often surrounded by a pale halo.2 In August and September 2002, 9 adult patients (7 men and 2 women; age range, 34-68 years) from an asylum for the chronically mentally ill in Parma, Italy, were referred to our institution for acute onset of papules clinically resembling EPA lesions (Figure 1). These blanched by vitro pressure and showed a somewhat symmetric distribution. The most affected sites were the extremities; less commonly involved were the face and the trunk. Neither prodromal symptoms nor fever were observed. Clinical evaluation of all patients, including analyses of lymph nodes, spleen, and liver, revealed no abnormality. No immunosuppressive abnormalities or systemic disorders were found. Interestingly, 2 of the researchers who worked in close contact with the patients complained of the rapid appearance of EPA lesions during this period. A histologic study from 3 cutaneous lesions of the patients revealed, under a normal- looking epidermis, dilated small blood vessels in the superficial dermis that showed plump endothelial cells (Figure 2). Extensive laboratory tests for viral agents were performed (Table), and findings were all negative but for polymerase chain reaction detection of Epstein-Barr virus (EBV) DNA in 4 patients’ leukocytes and the presence of anti-EBV IgG in 7 of the examined serum samples (Table). The cutaneous lesions faded spontaneously after a period ranging from 8 to 18 days without sequelae. However, 1 patient experienced an EPA relapse on both arms 20 days after the first presentation, and these lesions disappeared in 7 days.