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Skin ulcers and disseminated abscesses are characteristic of Serratia marcescens infection in older patients with chronic granulomatous disease

J. Friend, Dianne M Hilligoss, M. Marquesen, Jean Ulrick, Tyra Estwick, M. Turner, E. Cowen, V. Anderson et al.

Journal of Allergy and Clinical Immunology, 2009

15 participants · 22 citations

Abstract

To the Editor:

Chronic Granulomatous Disease (CGD) is a primary immune deficiency resulting from mutations in any of four subunits of phagocytic cell NADPH oxidase. CGD patients are at risk for infections with catalase-positive bacteria and fungi [1-3]. Serratia marcescens infection of bone and soft tissue is a common presentation of CGD in infancy [4-6]. In older children and adults with CGD, infection with Staphylococcus aureus, Burkholderia cepacia and Nocardia species, or fungi such as Aspergillus make up the majority of infections [3,4]. Our experience indicates S. marcescens remains a significant cause of infection in older children and adults with CGD. These infections have a pattern different from that seen in CGD infants. Osteomyelitis is less common, skin infections form poorly healing ulcers [7], and infections frequently occur at multiple sites.

We describe 16 episodes of S. marcescens infections in 15 patients 8–39 years of age with CGD followed between 1999–2008 at the National Institutes of Health (Table 1). Only patients with culture documented S. marcescens were included in this report. All patients were participants in an IRB approved study of the natural history of CGD. Written informed consent specifically allowed study of infections and other complications of CGD.

Table 1

CGD mutation, age at diagnosis of infection, clinical sites of infection and length of antibiotic therapy in patients presenting with S. marcescens infections

Three patients had…

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