Bacillary epithelioid angiomatosis

Introduction

Introduction to bacillary epithelioid hemangiopathy Bacillary Epithelioid Angiomatosis, also known as bacillary angiomorphosis or epithelioid angiomatosis, was first reported by Stoler in 1983. It found a multiple cutaneous vascular proliferative disease different from Kaposi sarcoma from an AIDS patient, and found a small bacillus in the skin lesion, which is considered to be a new infectious disease that causes the proliferation of small blood vessels in the skin and viscera. . basic knowledge The proportion of sickness: 0.01% Susceptible people: Occurs in male HIV-infected people Mode of infection: non-infectious Complications: sepsis bacteremia hepatitis

Cause

Causes of bacillary epithelioid angiomatosis

Infection (30%):

Electron microscopy of the lesions of the disease showed that there were small bacilli similar to cat scratching, the size was about 0.5m × 1.5m, and the bacilli were distributed in groups or bundled. At present, bacterial culture has been successfully reported, but in the end Which kind of bacteria belong to it is not clear, some researchers believe that it is related to the newly discovered caterpillar bacillus, even the cat scratch bacillus, only in the case of normal immune function of the patient, granulomatous damage occurs, and when there is immunity In the case of defects (such as HIV infection), it is manifested as vascular proliferative damage.

Immune Deficiency (30%):

The pathogenesis is still inaccurate, some researchers have observed; in the case of normal immune function of patients, granulomatous lesions occur, and in the case of immunodeficiency (such as HIV infection), it shows vascular proliferative damage.

Rickettsia-like microorganisms (20%):

There is also evidence that the pathogen causing the disease is a rickettsia or rickettsia-like microorganism, which is closely related to the bacillus Barton and the five-day rickettsia.

Prevention

Bacterial epithelioid hemangioma prevention

There is no effective preventive measure for this disease. Early detection and early diagnosis are the key to the prevention and treatment of this disease.

Complication

Bacterial epithelioid angiomatosis complications Complications septic bacteremia hepatitis

Disseminated multiple organ infections, sepsis-like syndrome, bacteremia, etc. in some cases, and hepatic involvement in purpuric hepatitis.

Symptom

Bacterial epithelioid hemangiomatosis symptoms Common symptoms Periosteal reaction HIV infection Gastrointestinal symptoms Sepsis papulosis Bacterial granulomatous vascular hyperplasia

Most of the disease occurs in the process of HIV infection, but it can also occur in cancer patients receiving chemotherapy. Organ transplanters rarely occur in normal people. Most cases are male, which may be more related to male HIV infection, 20%. Cases have a recent history of cat scratching. Skin damage is the most common symptom of this disease. It can also affect the liver, spleen, lung, bronchial mucosa, pleura, gastrointestinal mucosa, bone marrow, oral mucosa, conjunctiva and other organs.

Skin lesions can be divided into dermal and subcutaneous types. The dermal lesions are red or dark red needle-sized papules, which gradually enlarge into hemispherical, pointed or pedicled nodules, showing skin-like hardness, and can be ulcerated in the late stage. Serous exudation, the number can vary from single to multiple to hundreds of thousands, can occur anywhere in the body, a single skin lesion on the face or hand can be similar to purulent granuloma, after remission, leaving a slight pigment Sinking and induration, the larger the damage, the skin atrophy can occur, generally no symptoms, the subcutaneous type is a few centimeters in diameter plaque, often similar to cellulitis, common bone lesions under the skin lesions, can be prior to the lesion Bone damage mainly occurs in the distal part of the limb, often accompanied by pain. X-rays can be found as border-clear osteolytic lesions, or unclear margins with obvious cortical damage. Diffuse lesions are often accompanied by periosteal reactions.

Disseminated multiple organ infections are often accompanied by systemic symptoms such as fever, weight loss, etc. Individual cases may have sepsis-like syndrome, bacteremia, etc. Liver involvement manifests as purpuric hepatitis, gastrointestinal symptoms may occur, and the liver may be enlarged. Symptoms such as elevated serum aminotransferase and alkaline phosphatase. Liver biopsy shows that there are many blood-filled cysts in the liver, which damage the mucous-like matrix and agglomerated granular purple matter. Warthin-Starry staining and electron microscopic observation confirmed granular substances. For bacteria, the same bacteria as the bacteria in the skin lesions.

Both Kaposi's sarcoma and Kaposi's sarcoma are vascular proliferative diseases that are common in HIV-infected patients. Both have similarities in clinical manifestations and histopathology, but the treatments and prognosis are quite different, occurring in the face and A single lesion in the hand is clinically similar to purulent granuloma. Therefore, it is very important to correctly identify this disease with Kaposi sarcoma. Suppurative granuloma is very important. The diagnosis depends mainly on histopathological changes. In histopathology, this disease has The following characteristics: endothelial cells are cuboidal to the cavity, endothelial cells often have metastasis; inflammatory infiltration by neutrophils, often accompanied by eosinophils, obvious leukocyte fragmentation; visible in the interstitial Purple granular material mass, which is agglomerated by the pathogenic bacteria of the disease; the fibrous spacer component lacking purulent granuloma has a leaf-like appearance.

Examine

Examination of bacillary epithelioid angiomatosis

Liver involvement, elevated serum transaminase and alkaline phosphatase, liver biopsy Warthin-Starry staining and electron microscopy confirmed that the granular material was a bacterium.

X-rays can be found as border-clear osteolytic lesions, or unclear margins with significant cortical damage, and diffuse lesions often associated with periosteal reactions.

Histopathology: epidermis flattened, superficial lesions may appear similar to purulent granuloma, the epidermis on both sides prolonged and curved, small round blood vessels showed lobular hyperplasia, with a large number of endothelial cells protruding into the cavity, cytoplasm may appear Rich, closely-connected cuboidal endothelial cells, irregular vacuoles in some cytoplasm, endothelial cell-like appearance, deep nucleus staining, oval or pleomorphic, sometimes mitotic images, endothelial cells in the center of vascular leaflets Necrosis, a large number of neutrophils and eosinophils infiltrated between proliferating endothelial cells, accompanied by obvious leukocyte fragmentation, irregular purple granular granules visible in the interstitial, clearly identified by Warthin-Starry staining This clump is made up of bacilli.

Diagnosis

Diagnosis and identification of bacillary epithelioid angiomatosis

Identification with Kaposi sarcoma, suppurative granuloma is very important.

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