TY - JOUR
AU1 - Edwards, Morven S
AU2 - Chinen, Javier
AU3 - Dutta, Ankhi
AB - A 2-year-old boy presented in September 2020 with a 3-day history of fever associated with a “bug bite–like” lesion on his left knee that enlarged and, because of pain, progressed to non–weight bearing despite treatment for 2 days with clindamycin and mupirocin. His medical history was notable for development, at 3 months of age, of a perianal abscess requiring surgical drainage and development of an enlarged right cervical lymph node at 13 months of age that persisted for 5 months, despite antimicrobial treatment, and then ruptured spontaneously and resolved. He had been otherwise healthy with normal growth and development. At presentation, the child was afebrile, alert, and playful in bed. A 4 × 6 cm pustular lesion with central crusting and surrounding erythema was present on the left lateral knee. There was a 4 × 4 cm area of erythema without induration proximal to the knee lesion (Figure 1) and erythema in the left inguinal area. He had 2 discrete pustular lesions with surrounding erythema on the right thigh, each approximately 1 cm. There was full passive range of motion of the left knee. The remainder of the physical examination was normal. The white blood cell count was 12 580 cells/mm3 with 62% neutrophils, 25% lymphocytes, and 12% mononuclear cells. The C-reactive protein was 22.4 mg/dL (normal: <1.0 mg/dL) and the erythrocyte sedimentation rate was 77 mm/hour (normal: <20 mm/hour). Plain radiographs of the left knee were normal. Figure 1. Open in new tabDownload slide Image of the left lateral knee showing a pustular lesion with central crusting and surrounding erythema. There is a discrete area of erythema at the mid-thigh (arrow) and erythema in the inguinal area (not shown). Cultures were obtained from blood and purulent material from the left knee lesion. Gram stain of the skin lesion had few white blood cells, many red blood cells, and no visible organisms. Vancomycin was initiated. The next day, contrast-enhanced magnetic resonance imaging of the left knee showed thickening and hyper-enhancement of the dermis at the anterolateral aspect of the knee and left knee lateral distal femoral epiphyseal cartilage infectious chondritis but no bony involvement. On hospital day 3, the blood culture was sterile but the wound culture was growing gram-negative bacilli that appeared violet-black on blood agar (Figure 2). A single blood test confirmed the underlying diagnosis. Figure 2. Open in new tabDownload slide Image of the organism growing on a blood agar plate. What is the diagnosis? Diagnosis Chromobacterium violaceum infection in a toddler with chronic granulomatous disease. The wound culture grew Chromobacterium violaceum, a rare opportunistic, gram-negative facultative anaerobe that is catalase positive. Most strains produce violacein, an insoluble pigment that imparts a violet-black color to colonies on solid media under aerobic conditions. Many reports of C. violaceum infection are from Southeast Asia [1]. Infections acquired in the United States are from southeastern states, especially Georgia, Florida, South Carolina, and Louisiana, including southeast Texas, and occur in the summer months, from May to September, after exposure to contaminated soil or water. Local cellulitis, ulcers, or pustular lesions often precede evidence of dissemination with formation of multiple abscesses in visceral organs and the brain that may result in a fatal outcome [2]. Chromobacterium violaceum infection can be the defining manifestation of chronic granulomatous disease (CGD). This rare pathogen is particularly associated with and is highly virulent in patients with CGD [3]. Approximately one-third of patients from several cohorts who had C. violaceum infection also had CGD [1]. Vulnerability relates to the critical dependence in vivo of C. violaceum upon host generation of microbicidal reactive oxygen intermediates that are defective in CGD patients [4]. Although the infection can occur in otherwise healthy children, a child diagnosed as having C. violaceum infection should undergo evaluation for neutrophil dysfunction. Neutrophil defects other than CGD, such as glucose 6-phosphate dehydrogenase deficiency, have also occurred in association with the pathogen [5]. The initial presentation usually is fever and cellulitis at the site of a wound incurred in association with exposure to soil or water. Infection can remain localized to the wound or it can progress rapidly to present as septic shock, often in association with metastatic abscesses in the liver, spleen, and/or lungs and brain. Additional manifestations of infection can include pneumonia, lymphadenitis, or osteomyelitis, among others. Multiple sites of abscesses may occur [6]. Antibiotics with the greatest activity against C. violaceum include fluoroquinolones, tetracycline, trimethoprim-sulfamethoxazole, imipenem, and gentamicin [7]. Our patient’s isolate was susceptible to aminoglycosides, including amikacin, gentamicin, and tobramycin; to ciprofloxacin and levofloxacin; and to cefepime, meropenem, minocycline, piperacillin-tazobactam, and trimethoprim-sulfamethoxazole. Prolonged treatment, usually for at least 6–12 weeks, is important for resolution of infection without relapse, which may occur due to incomplete resolution of abscesses or from hidden septic foci [8]. Ultrasound revealed no abdominal abscesses in our patient. He received trimethoprim-sulfamethoxazole for 3 months and he recovered fully. Results of a dihydrorhodamine assay showed absent neutrophil oxidative burst activity, consistent with the diagnosis of CGD. Later genetic testing indicated that the child had the X-linked form of CGD. Chronic granulomatous disease is a rare primary immunodeficiency with an estimated prevalence of 1 in 200 000–250 000 live births in the United States, caused by defects in the nicotinamide adenine dinucleotide phosphate oxidase complex, which is critical for superoxide production [9]. The CGD defect leads to frequent and severe infections largely united by production of the enzyme catalase. The majority of infections in patients from North America with CGD are due to 5 pathogens, Staphylococcus aureus, Serratia spp, Burkholderia spp, Aspergillus spp, and Nocardia spp [10]. References 1. Lee PP-W , Lau Y-L. Endemic infections in Southeast Asia provide new insights to the phenotypic spectrum of primary immunodeficiency disorders. Asian Pac J Allergy Immunol 2013 ; 31 : 217 – 26 . Google Scholar PubMed OpenURL Placeholder Text WorldCat 2. Frawley AA , Powell L, McQuiston JR, Gulvik CA, Bégué RE. Necrotizing pneumonia caused by Chromobacterium violaceum: report of a rare human pathogen causing disease in an immunodeficient child. Am J Trop Med Hyg 2018 ; 99 : 164 – 7 . Google Scholar Crossref Search ADS PubMed WorldCat 3. Macher AM , Casale TB, Fauci AS. Chronic granulomatous disease of childhood and Chromobacterium violaceum infections in the southeastern United States. Ann Intern Med 1982 ; 97 : 51 – 5 . Google Scholar Crossref Search ADS PubMed WorldCat 4. Segal BH , Ding L, Holland SM. Phagocyte NADPH oxidase, but not inducible nitric oxide synthase, is essential for early control of Burkholderia cepacia and Chromobacterium violaceum infection in mice. Infect Immun 2003 ; 71 : 205 – 10 . Google Scholar Crossref Search ADS PubMed WorldCat 5. Shao P-L , Hsueh P-R, Chang Y-C, et al. Chromobacterium violaceum infection in children: a case of fatal septicemia with nasopharyngeal abscess and literature review. Pediatr Infect Dis J 2002 ; 21 : 707 – 9 . Google Scholar Crossref Search ADS PubMed WorldCat 6. Erdem G , Leber A. Eikenella, Pasteurella, and Chromobacterium species. In: Long SS, Prober CG, Fischer M, eds. Principles and Practice of Pediatric Infectious Diseases , 5th ed. Philadelphia, PA : Elsevier , 2018 : 859 – 63 . Google Scholar Crossref Search ADS Google Preview WorldCat COPAC 7. Harmon N , Mortensen JE, Robinette E, Powell EA. Pediatric bacteremia caused by Chromobacterium haemolyticum/Chromobacterium aquaticum. Diagn Microbiol Infect Dis 2016 ; 86 : 108 – 11 . Google Scholar Crossref Search ADS PubMed WorldCat 8. Alisjahbana B , Debora J, Susandi E, Darmawan G. Chromobacterium violaceum: a review of an unexpected scourge. Int J Gen Med 2021 ; 14 : 3259 – 70 . Google Scholar PubMed OpenURL Placeholder Text WorldCat 9. Prince BT , Thielen BK, Williams KW, et al. Geographic variability and pathogen-specific considerations in the diagnosis and management of chronic granulomatous disease. Pediatric Health Med Ther 2020 ; 11 : 257 – 68 . Google Scholar Crossref Search ADS PubMed WorldCat 10. Marciano BE , Spalding C, Fitzgerald A, et al. Common severe infections in chronic granulomatous disease. Clin Infect Dis 2015 ; 60 : 1176 – 83 . Google Scholar Crossref Search ADS PubMed WorldCat Author notes Potential conflicts of interest. The authors: No reported conflicts of interest. All authors have submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest. © The Author(s) 2022. Published by Oxford University Press for the Infectious Diseases Society of America. All rights reserved. For permissions, e-mail: journals.permissions@oup.com. This article is published and distributed under the terms of the Oxford University Press, Standard Journals Publication Model (https://academic.oup.com/journals/pages/open_access/funder_policies/chorus/standard_publication_model) © The Author(s) 2022. Published by Oxford University Press for the Infectious Diseases Society of America. All rights reserved. For permissions, e-mail: journals.permissions@oup.com.
TI - Skin Lesions in a Toddler 
JF - Clinical Infectious Diseases
DO - 10.1093/cid/ciab948
DA - 2022-10-29
UR - https://www.deepdyve.com/lp/oxford-university-press/skin-lesions-in-a-toddler-dd7I7Hunqj
SP - 1665
EP - 1667
VL - 75
IS - 9
DP - DeepDyve
ER -