Leterrell-Silver disease
Introduction
Introduction to Letrell-West's disease Letterer-Siwedisease is an acute differentiated histiocytosis with a small age of onset, multiple lesions often invaded, eye and eyelid involvement are rare, and the prognosis is poor. basic knowledge Sickness ratio: 0.0006%-0.001% Susceptible people: no special people Mode of infection: non-infectious Complications: suppurative otitis media
Cause
Cause of Letrell-Siewe's disease
(1) Causes of the disease
The cause is unknown and may be related to immune dysfunction.
(two) pathogenesis
The pathogenesis is unclear.
Prevention
Letrell-Siwe disease 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
Lytreil-West's disease complications Complications of suppurative otitis media
Suppurative otitis media, pulmonary infiltration or infection and bone marrow lesions can also affect the iris, ciliary body and uvea.
Symptom
Lytres-Siewe's disease symptoms Common symptoms Pimples dyspnea persistent fever Lymph node enlargement Diarrhea Thrombocytopenia Cyst abscess Eyelid closure Incomplete osteoporosis
Immature tissue cells proliferate in a large amount, causing damage to normal human tissues, especially skin and spleen, which can cause bleeding and necrosis. The course of disease is short, often 15 days to 2 years. The disease is fierce and difficult to control, typical cases For persistent fever, with suppurative otitis media, rash occurs, which can occur continuously or intermittently. It usually occurs in the skin of the chest and back, followed by the skin of the trunk and limbs. The lesion is usually located in the nipple layer of the skin and can also invade the epidermis, producing Pautrier micro. Abscess, clinically seen eczema and yellow lesions, can also appear dark red papules, may be caused by bleeding, central can also have keratinized sputum, some patients have seborrheic eczema-like rash, a small number of patients with papules Nodular rash, the skin leaves atrophy or scar after healing.
In most patients with pulmonary interstitial tissue cell infiltration or infection caused by cough, difficulty breathing, and involving the heart, lung function, alveolar fibers affected and broken, resulting in alveolar cysts, may lead to pneumothorax, gastrointestinal disorders, common diarrhea, liver Spleen and lymph nodes, skull, pelvis and long bone involvement, osteoporosis and destruction, resulting in the entire thickness of the bone inside and outside the plate defects, bone marrow lesions caused by anemia, white blood cells, thrombocytopenia, such as bone marrow lesions are very extensive, visible in the surrounding blood Nucleated red blood cells, a small number of patients with tibia involvement, eyelid tissue involvement caused by ocular protrusion, swelling of the eyelids, swelling of the conjunctival hemorrhage, and incomplete closure of the eyelids may also involve the iris, ciliary body and uvea.
Examine
Check of Letrell-West's disease
Blood routine examination to understand the number of blood cells, if necessary, bone marrow puncture examination, such as the increase of tissue cells in the bone marrow image has a suggestive value for disease diagnosis.
Most of the tissue cells in the lesion are immature tissue cells with a diameter of 10 to 25 m, a nucleus oval, a nuclear membrane, a slight dent or groove, and the chromatin is fine and rare, so some nuclei are empty. Bubble-like, cytoplasm is slightly alkaline, phagocytic lipids can be seen in the cytoplasm (Fig. 2). Multinucleated giant cells are scattered in the lesions, and their phagocytosis is not obvious. Eosinophils, lymphocytes and plasma can also be seen in the lesions. Cells, chest X-ray first understand the lung infiltration and infection, and exclude X-ray of the pneumothorax, skull, pelvis, whole body and eyelids, CT or MRI examination can understand the lesions.
Diagnosis
Diagnosis and identification of Letrell-West's disease
The patient is infant, fever, rash, liver and spleen and lymph nodes, and some patients have invasion of the tibia and soft tissues of the eye. For these cases, laboratory tests should be performed: the cells in the blood are generally normal, and the white blood cells of a few patients rise significantly. Up to 90,000 and visible nucleated red blood cells; thrombocytopenia, anemia, increased tissue cells in the bone marrow, and a large number of immature tissue cells in the skin lesion biopsy, scattered in multinucleated giant cells and mononuclear inflammatory cells. Provide direct evidence.
