Bacillary Angiomatosis 

  • Author: KoKo Aung, MD, MPH, FACP; Chief Editor: Burke A Cunha, MD   more...
 
Updated: Sep 6, 2011
 

Background

Bacillary angiomatosis (BA) is the vascular proliferative form of Bartonella infection. Bacillary angiomatosis was first described in 1983 in a patient infected with HIV.[1] The disease has since been described in patients following organ transplants and in immunocompromised persons. It is occasionally reported in immunocompetent patients. Initially, bacillary angiomatosis was called epithelioid angiomatosis because of its histologic appearance.

In 1990, Relman et al identified a visible but uncultivable bacillus from affected tissues of patients with bacillary angiomatosis using molecular methods.[2] They concluded that the unique 16S gene sequence associated with epithelioid angiomatosis belonged to a previously uncharacterized microorganism, most closely related to Rochalimaea quintana. Later, the same organism was recovered in specialized culture media. The gram-negative organism was later named Rochalimaea henselae, and, in 1993, Rochalimaea was reclassified under the genus Bartonella. Bartonella henselae and Bartonella quintana each have been cultured from and detected in bacillary angiomatosis tissues. Bacillary angiomatosis is the second-most-common angiomatous skin lesion in persons infected with HIV.

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Pathophysiology

B henselae and B quintana are small gram-negative rods in the family Bartonellaceae. Bartonella, Rickettsia, Ehrlichia, and Afipia species all are part of the alpha-2 subgroup of the Alphaproteobacteria.

Bacillary angiomatosis can affect almost any organ system, although it most commonly affects skin and subcutaneous tissue. Subcutaneous lesions may erode into underlying bones (ie, osseous bacillary angiomatosis), especially the tibia, fibula, and radius. Involvement of ribs and vertebrae has been described. Rarely, skeletal muscles may be involved, resulting in pyomyositis. Mucous membranes of the conjunctiva and upper airway and perineum (anus and penis) may be affected. Bacillary angiomatosis may be accompanied by disseminated visceral disease (peliosis), mainly in the liver (peliosis hepatis), spleen, and lymph nodes.

Other internal organs that may be involved include the brain, bone marrow, heart, lungs, pleura, larynx, oropharynx, tongue, esophagus, stomach, duodenum, colon, peritoneum, diaphragm, kidneys, adrenal glands, pancreas, uterine cervix, and vulva. Extrinsic compression of the common bile duct by enlarged peripancreatic, celiac, and portohepatic nodes has been reported.

The pathogenesis of bacillary angiomatosis includes early blood-borne dissemination of organisms. Bartonella organisms readily attach to and may enter erythrocytes. They avoid opsonization and host phagocytosis by unknown mechanisms and become persistent within the intravascular compartment. An angiogenic factor may be responsible for the vascular proliferation observed in patients with bacillary angiomatosis because a similar factor mediates vasoproliferation in verruca peruana, the second stage of Bartonella bacilliformis infection.

Cutaneous lesions result almost equally from B henselae and B quintana infections. However, subcutaneous and osseous lesions are usually caused by B quintana infection. Visceral involvement is almost exclusively caused by B henselae infection. Neurological disorders are associated more frequently with B quintana infection than with B henselae.

Domestic cats (Felis domesticus) are the reservoirs of B henselae, which may be transmitted via cat bites or scratches or, potentially, by bites from cat fleas (Ctenocephalides felis). Kittens are more frequently associated with transmission of B henselae than older cats. Humans appear to be the only reservoir of B quintana; the human body louse, Pediculus humanus, is the transmission vector.

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Epidemiology

Frequency

United States

The exact incidence of bacillary angiomatosis is not known. Cases of bacillary angiomatosis have been reported in almost all states, especially in Florida, Texas, New York, and northern California (San Francisco area), areas with a high frequency of HIV infection.

International

Bacillary angiomatosis is reported less commonly in Europe than in North America, which may imply that either diagnoses are missed or that Europe has a minimal reservoir of bacilli. Cases have also been reported in Africa, Peru, Argentina, Brazil,[3] Turkey,[4] and Australia.[5] In 2008, the first case of bacillary angiomatosis associated with B henselae was described in Thailand.[6]

In 2005, an epidemiologic study using serum samples from 253 patients with HIV infection from northern Greece showed that Bartonella infection is much more prevalent among individuals infected with HIV than among healthy individuals in the same area.[7]

Mortality/Morbidity

The exact mortality and morbidity of bacillary angiomatosis is unknown because the condition was initially described only recently.

Race

Approximately 40% of US patients with bacillary angiomatosis are white, 40% are black, and 20% are of Hispanic origin.

Sex

Approximately 90% of US patients with bacillary angiomatosis are men, probably because a disproportionate number of patients infected with HIV are also men.

Age

Bacillary angiomatosis is extremely rare in children but was reported in a 12-year-old boy with acute leukemia who was undergoing chemotherapy and in a 6-year-old immunocompetent girl.

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Contributor Information and Disclosures
Author

KoKo Aung, MD, MPH, FACP  Associate Professor, Department of Medicine, University of Texas Health Science Center at San Antonio; Adjunct Associate Professor of Public Health, University of Texas School of Public Health

KoKo Aung, MD, MPH, FACP is a member of the following medical societies: American College of Physicians

Disclosure: Nothing to disclose.

Coauthor(s)

Thwe T Htay, MD  Assistant Professor, Department of Medicine, University of Texas Health Science Center at San Antonio

Thwe T Htay, MD is a member of the following medical societies: American College of Physicians

Disclosure: Nothing to disclose.

Romeo Papica II  MD, Staff Physician Premier Physicians

Disclosure: Nothing to disclose.

Harvey Kantor, MD  Chief, Professor, Department of Internal Medicine, Division of Infectious Diseases, Texas Tech University Health Science Center

Harvey Kantor, MD is a member of the following medical societies: American College of Physicians, American Medical Association, American Society for Microbiology, Illinois State Medical Society, Infectious Diseases Society of America, New York Academy of Sciences, Royal Society of Medicine, and Sigma Xi

Disclosure: Nothing to disclose.

Hesham M Elgouhari, MD  Hepatology/Transplant Hepatology Fellow, Cleveland Clinic

Hesham M Elgouhari, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Medical Association, American Society of Transplantation, and Infectious Diseases Society of America

Disclosure: Nothing to disclose.

Specialty Editor Board

Gary L Gorby, MD  Associate Professor, Departments of Internal Medicine and Medical Microbiology and Immunology, Division of Infectious Diseases, Creighton University School of Medicine; Associate Professor of Medicine, University of Nebraska Medical Center; Associate Chair, Omaha Veterans Affairs Medical Center

Gary L Gorby, MD is a member of the following medical societies: Alpha Omega Alpha, American Medical Association, American Society for Microbiology, Infectious Diseases Society of America, and New York Academy of Sciences

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Joseph F John Jr, MD, FACP, FIDSA, FSHEA  Clinical Professor of Medicine, Molecular Genetics and Microbiology, Medical University of South Carolina College of Medicine; Associate Chief of Staff for Education, Ralph H Johnson Veterans Affairs Medical Center

Disclosure: Nothing to disclose.

Eleftherios Mylonakis, MD  Clinical and Research Fellow, Department of Internal Medicine, Division of Infectious Diseases, Massachusetts General Hospital

Eleftherios Mylonakis, MD is a member of the following medical societies: American Association for the Advancement of Science, American College of Physicians, American Society for Microbiology, and Infectious Diseases Society of America

Disclosure: Nothing to disclose.

Chief Editor

Burke A Cunha, MD  Professor of Medicine, State University of New York School of Medicine at Stony Brook; Chief, Infectious Disease Division, Winthrop-University Hospital

Burke A Cunha, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, and Infectious Diseases Society of America

Disclosure: Nothing to disclose.

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