Bacillary angiomatosis A48.8

Systemic infectious disease occurring predominantly in HIV-infected patients with < 200 CD4 cells/μl, but also in non-HIV-infected patients with immunosuppression (e.g. organ transplant patients) with a close etiological connection to cat scratch disease. See also Bartonellosis.

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

Bartonella henselae and Afipia felis are the causative agents of cat scratch disease, which generally heals spontaneously. Traumatic contacts with cats are described in only about 1/3 of cases. Cofactors such as HIV, CMV or EBV infections have been described.

Infected target cells (endothelial cells) induce release of growth factors (VEGF vascular endothelial growth factor) leading to endothelial cell proliferation.

Bartonella henselae is primarily the causative agent of bacillary angiomatosis a clinical picture that occurs primarily in immunosuppressed individuals (e.g. AIDS patients). The clinical picture is reminiscent of verruga peruviana, a chronic form of the Andean endemic Oroya fever(Bartonella bacilliformis).

Integument: Usually painless skin lesions without itching. In addition to small papular, red, sometimes haemorrhagic skin lesions, multiple reddish-purple, cutaneous and subcutaneous tumours up to the size of a walnut are also found, sometimes with central ulceration. Fusion with fascia and bone is possible. The consistency of the nodules is rubbery solid! Three cutaneous forms of manifestation are observed:

• Subcutaneous, partly caked, round-oval, usually skin-colored tumors of variable size.
• Exophytic growing, reddish, often centrally ulcerated and crusted nodules that bleed easily when traumatized (resemble granuloma teleangiectaticum).
• Dry, hyperpigmented, hyperkeratotic plaques over osteolytic foci. V.a. observed in Africans.

General symptoms typically include fever, night sweats, and weight loss. Mucosal involvement is possible.

Systemic manifestations: Infestation of internal organs, e.g. liver, spleen, bones and CNS has been described. Formation of multiple cystic cavities in the liver(bacillary peliosis hepatis) is possible. Painful osteolysis of the distal extremities, liver involvement.

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.

• Erythromycin 4 times/day 500 mg p.o. for at least 8 weeks is the agent of choice.
• Alternatively: Doxycycline 2 times/day 100 mg p.o. for 8 weeks or longer. Alternatively: Azithromycin, Roxithromycin, Ciprofloxacin.

Cave! Jarisch-Herxheimer reaction!

Diagnosis of bacillary angiomatosis

1. Hof H et al (2019) Bartonella . In: Hof H, Schlüter D, Dörries R, eds Duale Reihe Medizinische Mikrobiologie. 7th, completely revised and expanded edition. Stuttgart: Thieme p 465-466
2. Parinaud H (1889) Conjonctivite infectieuse transmise par les animaux. Ann Oculistique 101: 252-253
3. Plettenberg A et al (1995) Bacillary angiomatosis. Dermatologist 46: 39-43
4. Sanders A, Kaliebe T, Bredt W (1996) Bartonella (Rochalimeae) infections: cat scratch disease and bacillary angiomatosis. DMW 121: 65-69
5. Stoler MH et al (1983) An atypical subcutaneous infection associated with acquired immune deficiency syndrome. Am J Clin Pathol 80: 714-8.
6. Wagemann B et al (2002) Unusual expression of a bacillary angiomatosis. Z Hautkr 77: 496-505