fatal midline granuloma
Introduction
Introduction to fatal midline granuloma Lethal midline granulomatosis (lethalmidline granuloma) is a destructive non-specific granulomatous ulcer of the mid-face tissue of unknown cause, which is chronic progressive and has a poor prognosis. First reported by Stewart in 1944, also known as facial granuloma granuloma, progressive lethal granuloma, gangrenous rhinitis. basic knowledge The proportion of illness: 0.002% Susceptible people: no specific population Mode of infection: non-infectious Complications: septic shock
Cause
Fatal midline granuloma
Cause:
The cause is still unclear. It may be a localized Wegener granulomatosis, or a manifestation of reticulocyte sarcoma. Some scholars believe that the pathogenesis is related to autoimmunity.
Pathogenesis:
The pathogenesis is not well understood, it may be a localized Wegener granulomatosis, or a manifestation of reticulocyte sarcoma, which may be related to autoimmunity.
Prevention
Fatal midline granuloma prevention
It is a destructive non-specific granulomatous ulcer of the central tissue of unknown cause, which is chronic progressive and has a poor prognosis.
Complication
Fatal midline granuloma complications Complications septic shock
The end of the failure period is due to major bleeding or secondary infection.
Symptom
Fatal midline granuloma symptoms Common symptoms Nasal nasal mucosa ulcer nasal mucosa can be granules... Sneezing nosebleeds high heat mucosa dry secondary infection bone destruction
More common in men aged 20 to 50, good in the middle of the face, especially the nose, is a progressive development, roughly divided into the following three.
1. From the beginning of the prodromal period, it is a nasal rhinorrhea-like symptom such as nasal congestion, sneezing, running water or serosa-like sputum. Sometimes blood is found in the sputum. The nasal mucosa is dry and covered with yellow-brown sputum or shallow in the nasal vestibule and septum. ulcer.
2. During the development period, months or years after the prodromal period, the sputum gradually increases and becomes purulent or bloody. It can be covered with dark smear, smear, and stench. The nasal septum, turbinate, skull base and hard palate can be seen during examination. The soft palate tissue is destroyed and develops into the nasopharynx, paranasal sinus, internal hemorrhoids and eyelids, forming large tissue defects and bone destruction. The condition is severe, but there is little pain and no involvement of cervical lymph nodes.
3. The disease progresses further in the debilitating period. Due to large tissue necrosis defects, the nasopharynx and upper jaw are completely exposed, the odor is unpleasant, the fever is high, the weight loss is weak, and the cachexia phenomenon is caused, and the death is caused by major bleeding or secondary infection.
Examine
Fatal midline granuloma examination
Histopathology: early non-specific inflammation, vascular endothelial swelling, may be associated with thrombosis, luminal occlusion, inflammatory cell infiltration around the blood vessels, mainly lymphocytes, often mixed with plasma cells, also have more elongated or shuttle In the form of a tissue with a round or oval nucleus, necrosis can develop to the surrounding and deep tissues except for the ulcer area. With the development of the lesion, a small number of heterogeneous mononuclear cells infiltrate around the blood vessel, and the nucleus is large and the staining is deep. The form is irregular.
Diagnosis
Diagnosis and identification of fatal midline granuloma
Diagnostic criteria
At present, there is no specific method for diagnosis, which needs to be diagnosed by clinical course analysis, histopathological examination and bacteriological examination and other similar diseases. The following points can be used as a diagnostic reference:
1. Chronic progressive granulomatous ulcer that originates in the middle of the face.
2. Histopathological examination is chronic non-specific inflammatory granulomatous changes.
3. Early local tissue is badly damaged and generally good.
4. Local lymph nodes are generally not enlarged.
Differential diagnosis:
1. Wegener granulomatosis This disease also invades the upper respiratory tract, but does not wear facial soft tissue, and has lower airway inflammation and granulomatous vasculitis. There are many giant cells and no abnormal cells in histology.
2. Lymphoma-like granulomatosis This disease usually does not have the above-mentioned respiratory symptoms as the first symptom, and the abnormal mononuclear cells appear in the inflammatory infiltration, and there is also a change in vasculitis.
