Bacillary angiomatosis

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Bacillary angiomatosis

Tanya N. Basu, Chrystalla Macedo and Richard C.D. Staughton

Evidence Levels:  A Double-blind study  B Clinical trial ≥ 20 subjects  C Clinical trial < 20 subjects  D Series ≥ 5 subjects  E Anecdotal case reports

image

First described in 1983, bacillary angiomatosis (BA) is a vasculoproliferative disorder caused by the bacteria Bartonella henselae and Bartonella quintana (previously Rochalimaea spp.). It typically presents in profoundly immunocompromised patients (e.g., in advanced HIV infection, post transplant or during cytotoxic chemotherapy). Angiomatous papules, nodules, or plaques may arise in the skin or systemically in any organ including the bone, central nervous system, liver, where the condition is termed peliosis hepatis, and spleen (peliosis splenis).

Vascular proliferation may be due to an angiogenic factor produced by the Bartonella genus. B. henselae is transmitted through a cat scratch or bite; it also causes cat scratch disease. B. quintana is transmitted by the human body louse and also causes trench fever. Virtually identical localized cutaneous lesions to BA are seen in verruga peruana, which occurs in Peru due to the related Bartonella bacilliformis, transmitted by sandflies.

Management strategy

Prompt diagnosis of bacillary angiomatosis is essential to prevent dissemination, which can be fatal. Clinical suspicion should be aroused in the context of a low CD4 lymphocyte count (<100) or other immunosuppression, especially with a history of exposure to cats (harboring B. henselae) or body lice (which carry B. quintana). Cutaneous lesions can be superficial cherry-red round papules with an eroded surface, similar to pyogenic granulomas, or violaceous, lichenoid plaques or deep subcutaneous nodules. Single lesions have been reported in immunocompetent patients at inoculation sites whereas in the immunocompromised the entire body surface may be affected. Lesions can be mistaken for Kaposi sarcoma or in-transit metastatic amelanotic melanoma and other malignancies because of the highly vascular and erosive nature of the lesions. In advanced HIV infections, differentials include deep fungal infection, e.g., cryptococcosis and histoplasmosis. Patients with extracutaneous disease may or may not have skin signs and can present with vomiting, abdominal pain, and deranged liver function (peliosis hepatis) or pancytopenia and splenomegaly (peliosis splenis). Presentation can also include fever, lymphadenopathy, night sweats, endocarditis, and anemia.

Histology allows easy differentiation and shows a lobular proliferation of capillaries and venules, with swollen endothelial cells containing clumps of bacteria.

The response of bacillary angiomatosis to antibiotic treatment is usually dramatic, in contrast to the response of cat scratch disease. Our drugs of first choice are the macrolides (e.g., erythromycin 500 mg four times daily, azithromycin 500 mg daily, clarithromycin 500 mg twice daily); doxycycline 100 mg twice daily as an alternative; intravenous treatment may be needed in severe disease or with gastrointestinal intolerance. Their use is based on anecdotal experience in the absence of systematic trials. Current recommendations are that treatment should be continued for 3 months where there is skin disease only, and 4 months where there is bone/visceral involvement or peliosis hepatis. Should relapse occur on the above regimens, long-term prophylaxis with erythromycin or doxycycline may be indicated. In practice, however, the introduction of highly active anti-retroviral therapy (HAART) should reverse the immunocompromise state and thus alter the response to treatment, making long-term antibiotic less necessary (although BA has been reported during immune reconstitution during HAART). The patient should be evaluated for parenchymal and osseous disease prior to treatment and warned that a Jarisch–Herxheimer reaction may occur after the first few doses of antibiotic.

A wide variety of therapeutic agents are mentioned in the literature, but there is a lack of correlation between the in vitro and in vivo drug susceptibility of Bartonella spp., which reduces the usefulness of laboratory data. The picture is clouded further by the different response of Bartonella spp. to drugs in each of the diseases it causes.

Specific investigations

Culture of the fastidious Gram negative rods of Bartonella spp. is extremely difficult, requiring special media and prolonged incubation of up to 45 days; it is invariably negative if antibiotics have been given. Skin biopsy is the essential diagnostic tool and shows characteristic appearances on histology and Warthin–Starry silver stains, which shows the organism, as can electron microscopy. Species confirmation can be obtained by PCR. Reliance on serology in the immunosuppressed is hazardous, but the Centers for Disease Control (CDC) definition of a positive test is an indirect fluorescence assay (IFA) titer of over 1 : 64.

Bacillary angiomatosis and bacillary peliosis in patients infected with human immunodeficiency virus: clinical characteristics in a case–control study.

Mohle-Boetani JC, Koehler JE, Berger TG, LeBoit PE, Kemper CA, Reingold AL, et al. Clin Infect Dis 1996; 22: 794–800.

Forty-two cases were compared to 84 matched controls and the distinguishing clinical characteristics were evaluated. Significant differences included the presence of anemia (hematocrit <0.36), raised alkaline phosphatase and aspartate aminotransferase levels, and a low CD4 lymphocyte count (median being 21/mm3 compared to 186/mm3 in controls). There was no difference in the white blood cell count, creatinine, bilirubin, and alanine aminotransferase levels. Clinical signs included fever, abdominal pain, and lymphadenopathy.

First-line therapies

imageErythromycin C
imageAzithromycin C
imageClarithromycin C

Second-line therapies

imageDoxycycline C
imageTetracycline D
imageRifampin D

Third-line therapies

imageGentamicin E
imageThird- and fourth-generation cephalosporins E

[/level-membership-for-dermatology-category][not-level-membership-for-dermatology-category]

Bacillary angiomatosis

Tanya N. Basu, Chrystalla Macedo and Richard C.D. Staughton

Evidence Levels:  A Double-blind study  B Clinical trial ≥ 20 subjects  C Clinical trial < 20 subjects  D Series ≥ 5 subjects  E Anecdotal case reports

image

First described in 1983, bacillary angiomatosis (BA) is a vasculoproliferative disorder caused by the bacteria Bartonella henselae and Bartonella quintana (previously Rochalimaea spp.). It typically presents in profoundly immunocompromised patients (e.g., in advanced HIV infection, post transplant or during cytotoxic chemotherapy). Angiomatous papules, nodules, or plaques may arise in the skin or systemically in any organ including the bone, central nervous system, liver, where the condition is termed peliosis hepatis, and spleen (peliosis splenis).

Vascular proliferation may be due to an angiogenic factor produced by the Bartonella genus. B. henselae is transmitted through a cat scratch or bite; it also causes cat scratch disease. B. quintana is transmitted by the human body louse and also causes trench fever. Virtually identical localized cutaneous lesions to BA are seen in verruga peruana, which occurs in Peru due to the related Bartonella bacilliformis, transmitted by sandflies.

Management strategy

Prompt diagnosis of bacillary angiomatosis is essential to prevent dissemination, which can be fatal. Clinical suspicion should be aroused in the context of a low CD4 lymphocyte count (<100) or other immunosuppression, especially with a history of exposure to cats (harboring B. henselae) or body lice (which carry B. quintana). Cutaneous lesions can be superficial cherry-red round papules with an eroded surface, similar to pyogenic granulomas, or violaceous, lichenoid plaques or deep subcutaneous nodules. Single lesions have been reported in immunocompetent patients at inoculation sites whereas in the immunocompromised the entire body surface may be affected. Lesions can be mistaken for Kaposi sarcoma or in-transit metastatic amelanotic melanoma and other malignancies because of the highly vascular and erosive nature of the lesions. In advanced HIV infections, differentials include deep fungal infection, e.g., cryptococcosis and histoplasmosis. Patients with extracutaneous disease may or may not have skin signs and can present with vomiting, abdominal pain, and deranged liver function (peliosis hepatis) or pancytopenia and splenomegaly (peliosis splenis). Presentation can also include fever, lymphadenopathy, night sweats, endocarditis, and anemia.

Histology allows easy differentiation and shows a lobular proliferation of capillaries and venules, with swollen endothelial cells containing clumps of bacteria.

The response of bacillary angiomatosis to antibiotic treatment is usually dramatic, in contrast to the response of cat scratch disease. Our drugs of first choice are the macrolides (e.g., erythromycin 500 mg four times daily, azithromycin 500 mg daily, clarithromycin 500 mg twice daily); doxycycline 100 mg twice daily as an alternative; intravenous treatment may be needed in severe disease or with gastrointestinal intolerance. Their use is based on anecdotal experience in the absence of systematic trials. Current recommendations are that treatment should be continued for 3 months where there is skin disease only, and 4 months where there is bone/visceral involvement or peliosis hepatis. Should relapse occur on the above regimens, long-term prophylaxis with erythromycin or doxycycline may be indicated. In practice, however, the introduction of highly active anti-retroviral therapy (HAART) should reverse the immunocompromise state and thus alter the response to treatment, making long-term antibiotic less necessary (although BA has been reported during immune reconstitution during HAART). The patient should be evaluated for parenchymal and osseous disease prior to treatment and warned that a Jarisch–Herxheimer reaction may occur after the first few doses of antibiotic.

A wide variety of therapeutic agents are mentioned in the literature, but there is a lack of correlation between the in vitro and in vivo drug susceptibility of Bartonella spp., which reduces the usefulness of laboratory data. The picture is clouded further by the different response of Bartonella spp. to drugs in each of the diseases it causes.