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We report an unusual case of encephalotrigeminal angiomatosis in which the facial and oral angioma was bilateral, and several teeth were congenitally absent. The developmental nature of the anomaly is reviewed. Encephalotrigeminal angiomatosis is commonly referred to as the Sturge-Weber syndrome, after Sturge and Weber who first described this affliction in 1879. The main clinical features of this syndrome are 1. venous angiomatosis of the leptomeninges of the cerebral-cortex, usually unilaterally 2. ipsilateral facial angiomatosis that often follows in outline the distribution of the trigeminal nerve (Fig 1) 3. ipsilateral gyriform calcification of the cerebral cortex 4. epileptic convulsions (contralateral focus) or other seizures 5. ocular defects (choroidal angioma, glaucoma hemianopia) 6. mental retardation 7. contralateral hemiplegia 8. obesity 9. oral mucosal and gingival involvement. Other less typical features are 1. association with hypomelanosis of 1 to 10 2. leptomeningeal angioma contralateral to the facial nevus 3. leptomeningeal angioma without facial vascular naevus 4. association with gastro intestinal hemorrhage 5. paranasal sinus enlargement. This syndrome that affects males and females equally, is a rare congenital disorder, apparently hamartomatous in nature, from persistence of a primitive embryonal vascular plexus. During the sixth week of intra-uterine life this plexus develops around the cephalic portion of the neural tube and under the ectoderm in the region destined to become facial skin. In the Sturge-Weber syndrome, the vascular plexus fails to regress, as is normal during the ninth week, resulting in angiomatosis of the related tissues. Variation in the degree of persistence or regression of the vascular plexus accounts for unilaterality or bilaterality of involvement, and also for an incomplete syndrome in which the leptomeninges, but not the facial tissues are affected. Leptomeningeal angiomatosis is the primary abnormality of encephalotrigeminal angiomatosis, all other features of the syndrome probably being secondary to this. Calcification of the cortex is a poorly understood phenomenon which may result from stasis of blood in the angioma, associated with altered local metabolism. Epilepsy and other neurological seizures, and mental retardation are probably, in their turn, secondary to the cortical calcification. The most striking clinical feature of the Sturge-Weber syndrome is the facial vascular naevus which generally follows the distribution of innervation of one or more divisions of the trigeminal nerve, whence the term encephalotrigeminal angiomatosis. However, the naevus may be more extensive, down the neck and even onto the chest. The oral tissues underlying the affected facial tissues are invariably also angiomatous and may be considerably enlarged as a result. Alterations in eruption of teeth have also been noted. Histologically, affected soft tissues are very vascular, resembling a pyogenic granuloma or a capillary, or cavernous hemangioma. Yukna, Cassingham and Carr noted that affected bone was partially replaced by a delicate fibrous tissue containing thin-walled vascular spaces. Neither inflammatory cells, nor fatty or haemopoietic marrow was noted.
OBJECTIVES: To report seven cases of bacillary angiomatosis; to evaluate the most useful diagnostic tools; to analyse the clinical and epidemiological features associated with Bartonella quintana or Bartonella henselae infections. DESIGN: Clinical, diagnostic and epidemiological evaluation of 37 speciated bacillary angiomatosis cases in the literature, including the seven patients in our study. METHODS: Pathological examination of tissue samples, including Warthin-Starry staining and immunohistology; titre of antibodies to Bartonella sp.; detection of Bartonella sp. in blood and biopsy materials by culture or PCR; and statistical analysis of clinical and epidemiological features associated with B. quintana or B. henselae bacillary angiomatosis cases. RESULTS: Seven immunocompromised patients (six with AIDS and one patient with acute leukaemia) had bacillary angiomatosis confirmed by histology. B. quintana was cultured in three patients, whereas B. henselae DNA was amplified by PCR in the remaining four patients. Serum from only one patient reacted with Bartonella antigens. Amongst the 14 B. quintana and 23 B. henselae bacillary angiomatosis cases now reported in the literature, lymphadenopathies were significantly more frequent in B. henselae-infected patients, and neurological disorders of the central nervous system in B. quintana-infected patients. Risk factors were contact with cats, and homelessness or poor socioeconomic status in B. henselae and B. quintana bacillary angiomatosis cases, respectively. CONCLUSIONS: Although diagnosis of bacillary angiomatosis often remains solely based upon histology, culture or PCR-based methods are useful for the detection of Bartonella sp., and allow identification of the species involved, which is necessary to further characterize clinical and epidemiological features associated with B. quintana or B. henselae infections.
Fibrous long-spacing (FLS) collagen is a distinct ultrastructural form of collagen present in normal tissue, various tumors, and tissues degraded by bacterial collagenases in vivo and in vitro. An association between FLS collagen and bacillary angiomatosis has not been previously described. Six cases of bacillary angiomatosis, including one autopsy case with disseminated disease, were examined ultrastructurally. In addition, Kaposi sarcoma (3), pyogenic granuloma (3), capillary hemangioma (3), and cavernous hemangioma (2) were examined for comparison. A vascular proliferation in a lymph node from a patient with AIDS (1) and a case of pulmonary capillary hemangiomatosis (1), also in an AIDS patient, were studied. Abundant FLS collagen was identified in 4 of 6 cases of bacillary angiomatosis, in close association with the organisms. FLS collagen was not seen beyond the immediate vicinity of the organisms. The FLS collagen in bacillary angiomatosis was seen in skin biopsies and in lung and skeletal muscle in the autopsy case; in the latter case, as well as in the two AIDS-associated, nonbacillary angiomatosis, non-Kaposi sarcoma vascular proliferations, there was a striking distribution of FLS collagen around small blood vessels. Occasional FLS collagen was observed in all three pyogenic granuloma. When present in pyogenic granuloma, FLS collagen was intermixed with subendothelial collagen. Abundant FLS collagen was identified in close association with the organisms of bacillary angiomatosis in four cases; this morphologic alteration was seen in skin as well as lung and skeletal muscle. An association between FLS collagen and endothelial cells in normal tissue (Descemet's membrane) and in certain vascular proliferations appears to exist.
BACKGROUND: Bacillary angiomatosis is a clinicopathologic entity that most often is identified in the skin of patients with AIDS. This report presents an example of bacillary angiomatosis of the female genital tract. CASE: Bacillary angiomatosis presented as red-purple nodules of the vulva and cervix in a 32-year-old woman with AIDS. Histologic examination revealed the lobular epithelioid vascular proliferation and hazy clumps of bacteria that characterize bacillary angiomatosis. The diagnosis was confirmed on Warthin-Starry-stained issue and by blood cultures, which were positive for Bartonella (Rochalimaea) henselae. CONCLUSION: Accurate diagnosis of this infection is important because 1) bacillary angiomatosis is commonly mistaken for Kaposi sarcoma, 2) it is effectively treated with inexpensive antibiotics, and 3) undiagnosed and/or untreated bacillary angiomatosis may lead to overwhelming disseminated infection and death.
BACKGROUND: Bacillary angiomatosis is an infectious disease causing proliferation of small blood vessels in the skin and visceral organs of patients with human immunodeficiency virus infection and other immunocompromised hosts. The agent is often visualized in tissue sections of lesions with Warthin-Starry staining, but the bacillus has not been successfully cultured or identified. This bacillus may also cause cat scratch disease. METHODS: In attempting to identify this organism, we used the polymerase chain reaction. We used oligonucleotide primers complementary to the 16S ribosomal RNA genes of eubacteria to amplify 16S ribosomal gene fragments directly from tissue samples of bacillary angiomatosis. The DNA sequence of these fragments was determined and analyzed for phylogenetic relatedness to other known organisms. Normal tissues were studied in parallel. RESULTS: Tissue from three unrelated patients with bacillary angiomatosis yielded a unique 16S gene sequence. A sequence obtained from a fourth patient with bacillary angiomatosis differed from the sequence found in the other three patients at only 4 of 241 base positions. No related 16S gene fragment was detected in the normal tissues. These 16S sequences associated with bacillary angiomatosis belong to a previously uncharacterized microorganism, most closely related to Rochalimaea quintana. CONCLUSIONS: The cause of bacillary angiomatosis is a previously uncharacterized rickettsia-like organism, closely related to R. quintana. This method for the identification of an uncultured pathogen may be applicable to other infectious diseases of unknown cause.
BACKGROUND: Bacillary angiomatosis and bacillary peliosis are vascular proliferative manifestations of infection with species of the genus bartonella that occur predominantly in patients infected with the human immunodeficiency virus. Two species, B. henselae and B. quintana, have been associated with bacillary angiomatosis, but culture and speciation are difficult, and there has been little systematic evaluation of the species-specific disease characteristics. We studied 49 patients seen over eight years who were infected with bartonella species identified by molecular techniques and who had clinical lesions consistent with bacillary angiomatosis-peliosis. METHODS: In this case-control study, a standardized questionnaire about exposures was administered to patients with bacillary angiomatosis-peliosis and to 96 matched controls. The infecting bartonella species were determined by molecular techniques. RESULTS: Of the 49 patients with bacillary angiomatosis-peliosis, 26 (53 percent) were infected with B. henselae and 23 (47 percent) with B. quintana. Subcutaneous and lytic bone lesions were strongly associated with B. quintana, whereas peliosis hepatis was associated exclusively with B. henselae. Patients with B. henselae infection were identified throughout the study period and were epidemiologically linked to cat and flea exposure (P< or =0.004), whereas those with B. quintana were clustered and were characterized by low income (P=0.003), homelessness (P = 0.004), and exposure to lice (P= 0.03). Prior treatment with macrolide antibiotics appeared to be protective against infection with either species. CONCLUSIONS: B. henselae and B. quintana, the organisms that cause bacillary angiomatosis-peliosis, are associated with different epidemiologic risk factors and with predilections for involvement of different organs.
AIMS: Bacillary angiomatosis is a rare pseudoneoplastic angioproliferative lesion occurring in patients with AIDS. This condition has been associated with Bartonella henselae and Bartonella quintana infections. Human herpesvirus 8 (HHV-8) is thought to be the causative agent of Kaposi's sarcoma, a vasoproliferative neoplasm, also commonly found in patients with AIDS. The presence of HHV-8 in a cohort of patients with bacillary angiomatosis was investigated. METHODS: Eight cutaneous cases of biopsy confirmed bacillary angiomatosis were assessed for HHV-8 using standard solution phase polymerase chain reaction (PCR). RESULTS: No case of bacillary angiomatosis harboured HHV-8 DNA. CONCLUSIONS: HHV-8 was not demonstrated in the lesions of bacillary angiomatosis and therefore does not appear to play a role in the pathogenesis of this pseudoneoplastic angioproliferative disorder. This finding might be useful in the distinction of bacillary angiomatosis from Kaposi's sarcoma, because lesions from patients with Kaposi's sarcoma almost always contains HHV-8 DNA.
Since the recognition of the acquired immunodeficiency syndrome (AIDS) in 1981, previously rare infections and neoplasms have become increasingly common. Bacillary angiomatosis, undescribed in the medical literature prior to 1983, is now second in frequency only to Kaposi's sarcoma with respect to the cutaneous manifestations associated with human immunodeficiency virus (HIV) infection. Caused by Rochalimaea henselae, bacillary angiomatosis is easily treated, when diagnosed early, with erythromycin. We present two cases of bacillary angiomatosis that presented to Toronto General Hospital and review this new and clinically interesting entity. The incidence of bacillary angiomatosis will undoubtedly increase as the HIV epidemic accelerates. Since bacillary angiomatosis commonly affects the head and neck region, it is important for the otolaryngologist to become increasingly proficient in its diagnosis and treatment. The current AIDS crisis demands that the otolaryngologist become aware not only of bacillary angiomatosis, but also of the other cutaneous head and neck manifestations of HIV infection.
Bacillary angiomatosis is an opportunistic bacterial infection caused by either Bartonella henselae or B. quintana. The classic histologic presentation of bacillary angiomatosis involves three components: a lobular proliferation of capillaries with enlarged endothelial cells, neutrophilic debris, and clumps of finely granular material identified as bacteria with staining techniques. Pseudoepitheliomatous hyperplasia is a histologic reaction pattern characterized by epithelial proliferation in response to a variety of stimuli, including mycobacterial, fungal, and bacterial infections. We describe a case of bacillary angiomatosis associated with pseudoepitheliomatous hyperplasia in an immunocompromised patient with Acquired Immunodeficiency Syndrome. Histologic examination of a finger lesion demonstrated a capillary proliferation with neutrophilic debris and characteristic amorphous granular deposits. Warthin-Starry and Giemsa staining revealed clumps of coccobacilli. Cervical lymph node tissue also revealed organisms identified as Bartonella with PCR techniques. Stains and cultures for acid fast bacilli, fungus, and bacteria were negative. To our knowledge, there has been only one other report of bacillary angiomatosis presenting with pseudoepitheliomatous hyperplasia. We conclude that the differential diagnosis of entities associated with pseudoepitheliomatous hyperplasia should be expanded to include bacillary angiomatosis.
Bacillary angiomatosis, an infectious process associated with Rochalima spp., was thought until recently to be restricted to HIV-infected or otherwise immunosuppressed patients. In 1993, bacillary angiomatosis was reported in several immunocompetent adults. An extensive literature review failed to find references to bacillary angiomatosis in immunocompetent children. We describe a 6-year-old female who presented with a single, rapidly growing, friable, erythematous papule on her neck. Histologic examination of a biopsy specimen confirmed the diagnosis of bacillary angiomatosis. The patient was otherwise healthy, and her physical examination was normal. Laboratory studies, including HIV serology, were normal. The patient was treated with six weeks of oral erythromycin without evidence of recurrence. We present and discuss the implications of the first case of bacillary angiomatosis in an immunocompetent child.
The aim of this case series was to describe the clinical, laboratory and epidemiological characteristics and the presentation of bacillary angiomatosis cases (and/or parenchymal bacillary peliosis) that were identified in five public hospitals of Rio de Janeiro state between 1990 and 1997; these cases were compared with those previously described in the medical literature. Thirteen case-patients were enrolled in the study; the median age was 39 years and all patients were male. All patients were human immunodeficiency virus type 1 (HIV-1) infected and they had previous or concomitant HIV-associated opportunistic infections or malignancies diagnosed at the time bacillary angiomatosis was diagnosed. Median T4 helper lymphocyte counts of patients was 96 cells per mm(3). Cutaneous involvement was the most common clinical manifestation of bacillary angiomatosis in this study. Clinical remission following appropriate treatment was more common in our case series than that reported in the medical literature, while the incidence of relapse was similar. The frequency of bacillary angiomatosis in HIV patients calculated from two of the hospitals included in our study was 1.42 cases per 1000 patients, similar to the frequencies reported in the medical literature. Bacillary angiomatosis is an unusual opportunistic pathogen in our setting.
Human immunodeficiency virus (HIV)-associated bacillary angiomatosis has rarely been described in Africa. We report here the first case in Côte d'Ivoire. Although in industrialised countries bacillary angiomatosis has been described in patients with low CD4 count, this episode occurred in the first year following HIV-seroconversion in an adult patient with more than 500 CD4 cells per cubic millimetre. Symptoms rapidly and totally disappeared under erythromycin treatment, although with a relapse two years after the end of the first episode. In Africa where people living with HIV often present chronic cutaneous lesions, bacillary angiomatosis may be under-diagnosed. Bacillary angiomatosis must be systematically considered in face of lesions similar to Kaposi's sarcoma. Improving knowledge on symptoms of bacillary angiomatosis in Africa should lead to better treatment and a better estimation of its true frequency which may be underestimated.
Bacillary angiomatosis is a bacterial disease which affects mainly immunosuppressed patients. It may compromise any tissue, especially the skin, presenting papules, nodules or angiomatous tumors. We studied three young men with AIDS, all of them with 1-2 papules, nodules or subcutaneous tumors suggesting telangiectatic granuloma, sarcoma and lipoma. Microscopically, they were misdiagnosed as telangiectatic granuloma, Kaposi's sarcoma and "angioma with secondary inflammation". After reviewing the histopathology, we saw them to be composed by vessels with prominent endothelium and stroma rich in leukocytoclastic polymorphonuclears. Fibrinoid deposits were observed in the neighborhood of vessels as well as minute eosinophilic granular interstitial masses corresponding to Bartonella aggregates, criteria which answer to the diagnosis of bacillary angiomatosis with HE staining. The Warthin-Starry stain was not useful; using resin embedded tissue from paraffin-embedded material, bacterial clusters, both in semithin section stained with toluidine blue and in thin sections observed under the electron microscope, were clearly seen, confirming bacillary angiomatosis diagnosis. Patients were successfully treated with surgery and either erythromycin or doxycycline. We reviewed the entity as well as its differential diagnoses with telangiectatic granuloma, Kaposi's sarcoma, Carrión's disease, and cat-scratch disease. In conclusion, we showed the presence of bacillary angiomatosis in three patients, illustrated its typical histopathological appearance with HE staining and demonstrated the causal bacteria in thick sections and with the electron microscope. It is essential to recognize bacillary angiomatosis, as it can be cured with antibiotics.
BACKGROUND: Diffuse dermal angiomatosis is rare and usually considered a variant of reactive angioendotheliomatosis. It generally involves the extremities of patients with severe vascular disease and other comorbidities. Two patients with breast involvement have been described; however, neither had a relevant medical history or a vaso-occlusive disorder, but both had large pendulous breasts, and 1 was positive for IgM anticardiolipin and antinuclear antibodies. OBSERVATIONS: A 53-year-old woman had a reticulated, erythematous plaque with superficial ulceration and underlying tender nodules on her left breast. She had a history of cardiovascular disease and was a heavy smoker. Biopsy of the lesion showed diffuse proliferation of additional endothelial cells and small bland vessels within the papillary and upper reticular dermis. Angiography showed almost complete occlusion of the subclavian artery proximally. Diffuse dermal angiomatosis was diagnosed. With isotretinoin therapy, the lesions improved. One month later, after percutaneous subclavian arterial revascularization, the lesion resolved completely. A literature review suggested that a history of heavy smoking, in addition to a history of vascular disease, may be important in the pathogenesis of diffuse dermal angiomatosis. CONCLUSIONS: Clinical acumen is crucial to diagnose diffuse angiomatosis of the breast. Appropriate treatment to alleviate hypoxia may improve the patient's condition.