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Infectious Disease - Bacillary Angiomatosis/Peliosis Hepatis
DESCRIPTION
• A rare, vascular proliferative infectious disease affecting the skin and internal organs, predominantly observed in immunosuppressed persons, particularly those with T-cell deficits, such as HIV-positive patients. The name bacillary angiomatosis (BA) mostly refers to the cutaneous or disseminated form, whereas peliosis hepatica (PH) denotes the visceral form of this feverish sickness associated with Bartonella species.
EPIDEMIOLOGY
Prevalence This disease, encountered globally, is rare; isolated cases have been reported from various regions worldwide.
RISK FACTORS • HIV infection (CD4 <100) (1) • Other types of immunosuppression • Unsanitary settings, exposure to felines
COMPREHENSIVE PREVENTION
• Concerning cats for people who are HIV-positive or otherwise immunocompromised: It is advisable to acquire a cat that is over one year old and in good health. The patient must practice meticulous hand hygiene following the cleaning of the litter box. Prevent bites and scratches; wash hands immediately if they occur. Declawing or subjecting a cat to testing is not recommended. • Primary prophylaxis is not advised. Prophylactic administration of a macrolide provides protection against Bartonella infection. The potential requirement for secondary prophylaxis or the indefinite duration of treatment in HIV-positive patients remains undefined.
ETIOLOGY • Bartonella henselae (predominantly BA) and Bartonella quintana (mostly bacteremia). Bartonella species are gram-negative bacteria belonging to the alpha Proteobacteria family. • Infection with B. henselae is associated with cat exposure in individuals with HIV infection. Conversely, BA attributed to B. quintana is linked to body louse infection and homelessness.
DIAGNOSIS HISTORY
• The incubation period lasts a minimum of one week. • Clinical manifestations vary from isolated bacteremia to cutaneous and visceral (PH) disease, together with involvement of several organs (skin, bones, central nervous system). • If left untreated, it may be lethal. • BA: – Constitutional: Pyrexia, malaise, cachexia, anemia • PH: – Constitutional: Chronic fever, fatigue, weight reduction, stomach discomfort - Gastrointestinal manifestations: Nausea, emesis • Symptoms related to other organs or systems are contingent upon the individual body component involved (e.g., ostealgia, neurological impairments, etc.).
PHYSICAL EXAMINATION
• BA: – Dermatological symptoms (93% of patients): — Elevated, vivid red papules, ranging from one to hundreds in quantity and measuring from 1 mm to several centimeters in size, observed in two-thirds of patients. – Minor lesions may be obscured by a thinned epidermis, whereas bigger lesions are prone to erosion and hemorrhage. A peripheral collarette is prevalent. Subcutaneous nodular lesions, observed in one-fourth of patients, are typically big and may not exhibit any changes in the overlying skin. Cellulitic plaque-like lesions occur in 5–10% of patients and frequently overlay deeper osseous lesions. Ulcerations and folliculitis lesions are infrequent. Lesions can manifest in any region of the body; many types may develop simultaneously or in succession. - Extracutaneous signs primarily include bone and visceral lesions (liver, spleen), however several other organ/system involvements have been documented, with or without vascular proliferative alterations, painful or painless lymphadenopathy, and cerebral abscesses. Bone disease may initially present solely with pain and may or may not be accompanied by superficial skin sores. • PH: – Significant hepatomegaly, progressing over weeks or months – Splenomegaly – Potential involvement of the skin or other organs
DIAGNOSTIC TESTS AND INTERPRETATION
LAB
Diagnosis is established through the identification of causative organisms in hematoxylin and eosin-stained tissue sections (granular purple material); it may also be confirmed using Warthin-Starry or Brown–Hopps tissue stains. • Organisms manifest either solitarily or in clusters and entanglements. • Acquiring culture is challenging and labor-intensive. The organism can be extracted from the blood using lysis centrifugation cultures. Incubate for a minimum of 21 days. Polymerase chain reaction (PCR) is used for detection and species identification. • The sensitivity of serological tests is diminished in immunocompromised individuals. • Mildly raised transaminases (average ×2 of normal), moderate to severe elevation of alkaline phosphatase (average ×5 of normal), and normal or slightly higher bilirubin levels. Mild to moderate pancytopenia may manifest in the visceral form of the disease. Imaging • Bone disease: Standard bone radiographs reveal well-defined lytic lesions or indistinct areas of significant cortical destruction accompanied by pronounced periosteal response. • CT imaging may demonstrate hepatosplenomegaly and enlargement of intra-abdominal and/or retroperitoneal lymph nodes. Visceral parenchyma may exhibit a diverse consistency. Histopathological Observations • Biopsy or fine-needle aspiration specimens from the skin, liver, and lymph nodes are typically utilized. The pathological pattern is contingent upon the organ affected. • Skin: Lesions exhibit a "epithelioid hemangioma" look, with the presence of organisms demonstrated. • PH: Liver biopsy specimens exhibit significantly dilated, blood-filled cystic areas within the parenchyma. They are frequently linked to a myxoid stroma. Foci of necrosis are observable in advanced instances.
DIFFERENTIAL DIAGNOSIS • BA: – Kaposi’s sarcoma – Pyogenic granulomas – Angiomas – Verruga peruana (bartonellosis, prevalent in South America) • PH: – Kaposi’s sarcoma • Extracutaneous manifestations: – Additional lesions occurring in space – The potential coexistence with Kaposi’s sarcoma must constantly be contemplated.
THERAPY PHARMACEUTICALS
Initial Statement An initial assessment is required to ascertain the degree of organ involvement. Erythromycin 500 mg orally every 6 hours or doxycycline 100 mg every 12 hours (AII) • Treatment duration: BA 3 months; PH 4 months. Extended therapy for immunocompromised individuals. Patients may encounter a Jarisch–Herxheimer reaction and should get prophylactic antipyretic medications within the initial 72 hours of treatment. Second Line • In cases of intolerance to erythromycin or doxycycline, azithromycin or clarithromycin have demonstrated a clinical response (BIII). Combination therapy, incorporating rifampin (300 mg orally twice day) alongside either erythromycin or doxycycline, is advised for immunocompromised patients experiencing acute, life-threatening infections or central nervous system disorders. • TMP-SMX and ciprofloxacin have demonstrated variable clinical efficacy.
INPATIENT CONSIDERATIONS
Criteria for Admission Patients are often managed on an outpatient basis; however, inpatient care and intravenous antibiotics are required for those with significant skin disease, lytic bone lesions, visceral lesions, or fulminant disease.
CONTINUING CARE FOLLOW-UP SUGGESTIONS
• Following the commencement of treatment (about 4–7 days into therapy), substantial enhancement of cutaneous and visceral/other organ lesions is often observed. • Complete resolution typically occurs within 3 to 4 weeks. • Relapses manifest in around 15% of instances. • Treatment failure: Upon relapse, four months of uninterrupted suppressive therapy. Patient Surveillance Clinical monitoring is necessary. • Serial biochemical assays (e.g., liver function tests) may assist in evaluating the therapeutic response in visceral disease. Utilize x-rays or bone scans to assess bone pathology. • The potential for coexistence with Kaposi’s sarcoma should be explored if imaging results do not improve following adequate antibiotic therapy.
COMPLICATIONS
The visceral manifestation of the disease may be exacerbated by anemia, pancytopenia resulting from hypersplenism, and splenic rupture accompanied by hemoperitoneum.
DESCRIPTION
• A rare, vascular proliferative infectious disease affecting the skin and internal organs, predominantly observed in immunosuppressed persons, particularly those with T-cell deficits, such as HIV-positive patients. The name bacillary angiomatosis (BA) mostly refers to the cutaneous or disseminated form, whereas peliosis hepatica (PH) denotes the visceral form of this feverish sickness associated with Bartonella species.
EPIDEMIOLOGY
Prevalence This disease, encountered globally, is rare; isolated cases have been reported from various regions worldwide.
RISK FACTORS • HIV infection (CD4 <100) (1) • Other types of immunosuppression • Unsanitary settings, exposure to felines
COMPREHENSIVE PREVENTION
• Concerning cats for people who are HIV-positive or otherwise immunocompromised: It is advisable to acquire a cat that is over one year old and in good health. The patient must practice meticulous hand hygiene following the cleaning of the litter box. Prevent bites and scratches; wash hands immediately if they occur. Declawing or subjecting a cat to testing is not recommended. • Primary prophylaxis is not advised. Prophylactic administration of a macrolide provides protection against Bartonella infection. The potential requirement for secondary prophylaxis or the indefinite duration of treatment in HIV-positive patients remains undefined.
ETIOLOGY • Bartonella henselae (predominantly BA) and Bartonella quintana (mostly bacteremia). Bartonella species are gram-negative bacteria belonging to the alpha Proteobacteria family. • Infection with B. henselae is associated with cat exposure in individuals with HIV infection. Conversely, BA attributed to B. quintana is linked to body louse infection and homelessness.
DIAGNOSIS HISTORY
• The incubation period lasts a minimum of one week. • Clinical manifestations vary from isolated bacteremia to cutaneous and visceral (PH) disease, together with involvement of several organs (skin, bones, central nervous system). • If left untreated, it may be lethal. • BA: – Constitutional: Pyrexia, malaise, cachexia, anemia • PH: – Constitutional: Chronic fever, fatigue, weight reduction, stomach discomfort - Gastrointestinal manifestations: Nausea, emesis • Symptoms related to other organs or systems are contingent upon the individual body component involved (e.g., ostealgia, neurological impairments, etc.).
PHYSICAL EXAMINATION
• BA: – Dermatological symptoms (93% of patients): — Elevated, vivid red papules, ranging from one to hundreds in quantity and measuring from 1 mm to several centimeters in size, observed in two-thirds of patients. – Minor lesions may be obscured by a thinned epidermis, whereas bigger lesions are prone to erosion and hemorrhage. A peripheral collarette is prevalent. Subcutaneous nodular lesions, observed in one-fourth of patients, are typically big and may not exhibit any changes in the overlying skin. Cellulitic plaque-like lesions occur in 5–10% of patients and frequently overlay deeper osseous lesions. Ulcerations and folliculitis lesions are infrequent. Lesions can manifest in any region of the body; many types may develop simultaneously or in succession. - Extracutaneous signs primarily include bone and visceral lesions (liver, spleen), however several other organ/system involvements have been documented, with or without vascular proliferative alterations, painful or painless lymphadenopathy, and cerebral abscesses. Bone disease may initially present solely with pain and may or may not be accompanied by superficial skin sores. • PH: – Significant hepatomegaly, progressing over weeks or months – Splenomegaly – Potential involvement of the skin or other organs
DIAGNOSTIC TESTS AND INTERPRETATION
LAB
Diagnosis is established through the identification of causative organisms in hematoxylin and eosin-stained tissue sections (granular purple material); it may also be confirmed using Warthin-Starry or Brown–Hopps tissue stains. • Organisms manifest either solitarily or in clusters and entanglements. • Acquiring culture is challenging and labor-intensive. The organism can be extracted from the blood using lysis centrifugation cultures. Incubate for a minimum of 21 days. Polymerase chain reaction (PCR) is used for detection and species identification. • The sensitivity of serological tests is diminished in immunocompromised individuals. • Mildly raised transaminases (average ×2 of normal), moderate to severe elevation of alkaline phosphatase (average ×5 of normal), and normal or slightly higher bilirubin levels. Mild to moderate pancytopenia may manifest in the visceral form of the disease. Imaging • Bone disease: Standard bone radiographs reveal well-defined lytic lesions or indistinct areas of significant cortical destruction accompanied by pronounced periosteal response. • CT imaging may demonstrate hepatosplenomegaly and enlargement of intra-abdominal and/or retroperitoneal lymph nodes. Visceral parenchyma may exhibit a diverse consistency. Histopathological Observations • Biopsy or fine-needle aspiration specimens from the skin, liver, and lymph nodes are typically utilized. The pathological pattern is contingent upon the organ affected. • Skin: Lesions exhibit a "epithelioid hemangioma" look, with the presence of organisms demonstrated. • PH: Liver biopsy specimens exhibit significantly dilated, blood-filled cystic areas within the parenchyma. They are frequently linked to a myxoid stroma. Foci of necrosis are observable in advanced instances.
DIFFERENTIAL DIAGNOSIS • BA: – Kaposi’s sarcoma – Pyogenic granulomas – Angiomas – Verruga peruana (bartonellosis, prevalent in South America) • PH: – Kaposi’s sarcoma • Extracutaneous manifestations: – Additional lesions occurring in space – The potential coexistence with Kaposi’s sarcoma must constantly be contemplated.
THERAPY PHARMACEUTICALS
Initial Statement An initial assessment is required to ascertain the degree of organ involvement. Erythromycin 500 mg orally every 6 hours or doxycycline 100 mg every 12 hours (AII) • Treatment duration: BA 3 months; PH 4 months. Extended therapy for immunocompromised individuals. Patients may encounter a Jarisch–Herxheimer reaction and should get prophylactic antipyretic medications within the initial 72 hours of treatment. Second Line • In cases of intolerance to erythromycin or doxycycline, azithromycin or clarithromycin have demonstrated a clinical response (BIII). Combination therapy, incorporating rifampin (300 mg orally twice day) alongside either erythromycin or doxycycline, is advised for immunocompromised patients experiencing acute, life-threatening infections or central nervous system disorders. • TMP-SMX and ciprofloxacin have demonstrated variable clinical efficacy.
INPATIENT CONSIDERATIONS
Criteria for Admission Patients are often managed on an outpatient basis; however, inpatient care and intravenous antibiotics are required for those with significant skin disease, lytic bone lesions, visceral lesions, or fulminant disease.
CONTINUING CARE FOLLOW-UP SUGGESTIONS
• Following the commencement of treatment (about 4–7 days into therapy), substantial enhancement of cutaneous and visceral/other organ lesions is often observed. • Complete resolution typically occurs within 3 to 4 weeks. • Relapses manifest in around 15% of instances. • Treatment failure: Upon relapse, four months of uninterrupted suppressive therapy. Patient Surveillance Clinical monitoring is necessary. • Serial biochemical assays (e.g., liver function tests) may assist in evaluating the therapeutic response in visceral disease. Utilize x-rays or bone scans to assess bone pathology. • The potential for coexistence with Kaposi’s sarcoma should be explored if imaging results do not improve following adequate antibiotic therapy.
COMPLICATIONS
The visceral manifestation of the disease may be exacerbated by anemia, pancytopenia resulting from hypersplenism, and splenic rupture accompanied by hemoperitoneum.
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