eMedicine Specialties > Infectious Diseases > Skin and Soft-Tissue Infections

Bacillary Angiomatosis

Author: KoKo Aung, MD, MPH, FACP, Assistant Professor, Department of Medicine, University of Texas Health Science Center; Adjunct Assistant Professor of Public Health, University of Texas School of Public Health
Coauthor(s): Thwe T Htay, MD, Assistant Professor, Department of Medicine, University of Texas Health Science Center at San Antonio; Romeo Papica II, MD, Research Associate, Department of Internal Medicine, Texas Tech University Health Sciences Center; Harvey Kantor, MD, Chief, Professor, Department of Internal Medicine, Division of Infectious Diseases, Texas Tech University Health Science Center; Hesham M Elgouhari, MD, Hepatology/Transplant Hepatology Fellow, Cleveland Clinic
Contributor Information and Disclosures

Updated: Aug 13, 2008

Introduction

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.

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.

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, and Argentina.

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.³

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.

Clinical

History

• Most patients with bacillary angiomatosis are infected with HIV and have CD4⁺ cell counts of less than 200/µL.
• The duration of symptoms before diagnosis is usually several months.
• Features of skin, subcutaneous, mucosal, and osseous lesions caused by bacillary angiomatosis include the following:
  • Raised red or purple lesions in the skin that bleed when traumatized
  • Similar lesions in the oral mucosa, tongue, oropharynx, nose, penis, or anus
  • Bone pain, frequently in the forearms or legs
• Visceral involvement associated with bacillary angiomatosis may be asymptomatic or may cause the following symptoms:
  • Fever, chills, malaise, night sweats, anorexia, and weight loss
  • Abdominal pain, nausea, and vomiting (peliosis hepatis)
  • Jaundice secondary to biliary obstruction caused by external compression of periportal lymph nodes
  • Intra-abdominal mass and gastrointestinal bleeding
  • Abdominal cramps, tenesmus, and bloody diarrhea (colonic bacillary angiomatosis)
  • Psychiatric symptoms, such as exacerbation of depression or new-onset psychosis; personality changes, including anxiety and irritability; headache; trigeminal neuralgia; seizures; or back pain (CNS bacillary angiomatosis)
  • Difficulty breathing secondary to laryngeal obstruction
• Underlying disease conditions may include the following:
  • Commonly, a history of HIV infection, organ transplantation, leukemia, or chemotherapy
  • Bacillary angiomatosis developing prior to HIV seroconversion in some patients
  • Apparent immunocompetence in some patients
• Bacillary angiomatosis was reported in a patient who was HIV-seronegative but had idiopathic thrombocytopenic purpura, had undergone splenectomy, and had been administered long-term systemic prednisone.⁴ Another recent report described an immunocompetent child with infected facial wound, in the vicinity of which bacillary angiomatosis lesions had developed. Similar lesions also appeared at the donor site of the skin graft, which was grafted on the facial wound.⁵ Multiple leg ulcers caused by bacillary angiomatosis without a history of direct contact with cats in an adult immunocompetent man has also been reported.⁶

Physical

• Skin and subcutaneous lesions  
  • Cutaneous lesions due to bacillary angiomatosis may take one of the following forms: (1) solitary or multiple red, purple, flesh-colored, or colorless papules (hemangiomalike lesions) varying in size from 1 mm to several centimeters; (2) nodules, often covered with a fine tightly adherent scale; (3) large, friable, pedunculated, or polypoid exophytic masses; or (4) hyperpigmented, hyperkeratotic, indurated plaques, typically on extremities, often overlying osseous defects.
  • The number of lesions may vary from 1 to more than 1000, and they are often multiple. The lesions on patients with multiple lesions often demonstrate more than one morphological appearance. Black patients, in particular, may bear the plaque form.
  • Cutaneous lesions may develop ulceration, discharge, and crusting and are often tender. Smaller lesions tend to be covered with an attenuated epidermis, while larger lesions tend to erode and bleed. Most lesions are rubbery and firm upon palpation and are usually freely mobile. They may be associated with regional lymphadenopathy. Lesions may regress spontaneously, but this is rare.
  • Subcutaneous nodules may erode through the surface and become friable and superinfected. Deep lesions are usually uncolored and either mobile or fixed to the underlying tissues. They are often tender. The overlying skin may appear normal.
• Mucosal lesions: These are similar to other lesions and may involve oral, conjunctival, nasal, anal, or penile mucosal surfaces.
• Visceral involvement  
  • Visceral involvement may lead to fever, abdominal distension, hepatomegaly, and splenomegaly. This involvement may eventually progress to bacteremia and sepsis syndrome.
  • Neurological deficits may accompany intracranial mass lesions.
  • Visceral involvement may occur in the absence of cutaneous lesions. In this case, the diagnosis is often delayed because the manifestations of visceral involvement are nonspecific.
  • A recent retrospective analysis of 37 speciated bacillary angiomatosis cases demonstrated that fever was present in two thirds of the patients and weight loss in one third of the patients, including patients without extracutaneous involvement.⁷

Causes

• Risk factors for bacillary angiomatosis include the following:
  • HIV infection
  • Chronic lymphocytic leukemia
  • Cytotoxic chemotherapy
  • Organ transplantations
• Additional risk factors for bacillary angiomatosis associated with B henselae infection include the following:
  • Cat ownership
  • Cat bites
  • Cat scratches
• Additional risk factors for bacillary angiomatosis associated with B quintana infection include the following:
  • Homelessness
  • Low socioeconomic status
  • Exposure to body and hair lice

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