Angiomatosis bacillary A48.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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History
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Parinaud, 1889; Stoler, 1983

Definition
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Predominantly in HIV-infected patients with < 200 CD4 cells/μl, but also in non-HIV-infected patients with immunosuppression (e.g. organ transplants), infectious disease with close etiological connection to cat scratch disease. S.a.u. Bartonellosis.

Pathogen
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Bartonella henselae and, more rarely, Bartonella quintana (small pleomorphic bacteria; see below Bartonellose); vectors in Bartonella henselae are cats and cat fleas; in Bartonella quintana the vector is the clothes louse (see below Pediculosis corporis).

Etiopathogenesis
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B. henselae and B. quintana are identical with the causative agents of cat scratch disease. Traumatic contacts to cats are only described in 1/3 of the cases. Cofactors like HIV, CMV or EBV have been described. Affected target cells (endothelial cells) induce release of growth factors (VEGF vascular endothelial growth factor), which lead to endothelial cell proliferation.

Clinical features
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  • Integument: Usually painless skin changes without itching. In addition to small papular, red and sometimes hemorrhagic skin lesions, multiple reddish-violet, cutaneous and subcutaneous tumours up to walnut size are found, sometimes with central ulceration. Bonding with fascia and bones is possible. The consistency of the nodes is rubbery solid! Three cutaneous forms of manifestation are observed:
    • Subcutaneous, partly caked, round-oval, usually skin-coloured tumours of variable size.
    • Exophytically growing, reddish, often centrally ulcerated and encrusted, easily bleeding when traumatised (similar to the granuloma teleangiectaticum).
    • Dry, hyperpigmented, hyperkeratotic plaques over osteolytic foci. Mainly observed in Africans.
  • Typically, general symptoms such as fever, night sweats and weight loss occur. Infection of the mucosa is possible.
  • Systemic manifestations: Infestation of internal organs, e.g. liver, spleen, bones and CNS is described. Formation of multiple cystic cavities in the liver (bacillary peliosis hepatis) is possible. Painful osteolysis of the distal extremities, liver involvement.

Histology
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Lobular vascular proliferation of differently shaped blood vessels with predominantly clumsy, partly cubic or epithelioid endothelium. Inflammatory infiltrate with numerous neutrophils. Moderate nuclear atypia. Interstitially homogeneous or granulated eosinophilic material, multiple aggregated extracellular bacteria present, which can be detected in the affected tissue by means of Warthin-Starry staining or electron microscopy.

Diagnosis
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PCR in lesions, antibody determination (EIA: 88% positive), blood culture (in febrile state), cultivation from lymph nodes, see Table 1.

Differential diagnosis
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Granuloma teleangiectaticum, Kaposi's sarcoma, hemangiomas, dermatofibromas, papular exanthema of other genesis; various subcutaneous tumours; ulcerative pyodermias; syphilis II or III; verruga peruana (especially histological analogy), see below Oroya fever.

Complication(s)
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Peliosis hepatis can occur sporadically and rather rarely but more frequently in HIV-infected persons. These are vascular proliferations and cystic blood accumulations in the liver and/or spleen.

Therapy
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Antibiotic therapy should also be applied to single herds! In case of recurrence: lifelong therapy may be necessary!

External therapy
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For single lesions: excision, electrodissection, curettage or cryotherapy.

Internal therapy
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Cave! Jarisch-Herxheimer reaction!

Progression/forecast
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Complete healing under therapy, persistence of hyperpigmentation possible.

Tables
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Diagnosis of bacillary angiomatosis

Histology

Lobular proliferations of small blood vessels with clumpy endothelial cells protruding into the lumen (here: mitoses and atypia). Perivascular predominantly neutrophilic infiltrate with nuclear debris, interstitially granular material in cell dense areas (Whartin-Starry staining: bacterial cluster!).

Immunohistochemistry

Expression of endothelial (factor VIII) and histiocytic (alpha-1-antichymotrypsin) markers

Electron Microscopy

bacteria with typical 3-layer wall structure

Cultivation (cooking blood agar, 35 °C, 5%CO2 atmosphere, 2-6 weeks)

pinhead-sized, heterogeneous, whitish autoadherent colonies

Gram staining

Fine, slightly curved gram-negative rods

Gas Chromatography

Determination of cellular fatty acids

SDS Disc Electrophoresis

Cell membrane proteins

PCR amplification

DNA restriction endonuclease pattern, DNA hybridization and sequencing of the 16S rRNA gene

Literature
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  1. Parinaud H (1889) Conjonctivite infectieuse transmise par les animaux. Ann Oculistique 101: 252-253
  2. Plettenberg A et al (1995) Bacillary angiomatosis. Dermatologist 46: 39-43
  3. Sanders A, Kaliebe T, Bredt W (1996) Bartonella (Rochalimeae) infections: cat scratch disease and bacillary angiomatosis. DMW 121: 65-69
  4. Stoler MH et al (1983) An atypical subcutaneous infection associated with acquired immune deficiency syndrome. Am J Clin Pathol 80: 714-8
  5. Wagemann B et al (2002) Unusual expression of bacillary angiomatosis. Z Hautkr 77: 496-505

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

Authors

Last updated on: 29.10.2020