Hand-Hüller-Christian disease

Introduction to Hande-Schüller-Kesi Hand-Schüller-Christiandisease, also known as subacute or chronically differentiated histiocytosis, is more likely to cause bone destruction than acute differentiated histiocytosis, but better than Letterer- The prognosis of Siwe disease is good. Can cause right heart failure, pituitary or hypothalamic involvement and diabetes insipidus. basic knowledge The proportion of illness: 0.001% Susceptible people: children who are more than 3 years old Mode of infection: non-infectious Complications: ulcer bleeding

The cause of Hande-Schüller-Kesi

Causes:

The cause is unknown, may be related to immune dysfunction, the disease is more common in children over 3 years old, adults are rare, the complications of the disease in the late stage of the disease are mostly diabetes insipidus.

Pathogenesis:

The pathogenesis is unclear. The patient's face, eyelids, trunk, perineum and underarm skin ulcers or yellow tumors, oral mucosal ulcers, hilar and pulmonary interstitial fibrosis due to infiltration of tissue cells and inflammatory cells.

Hande-Schüller-Kesi disease prevention

1, mostly based on light food, pay attention to the law of diet.

2. Eat properly according to the doctor's advice.

3, the disease does not have too much taboo on the diet, a reasonable diet can be.

Hande-Schüller-Kesi disease complications Complications ulcer disease bleeding

Multiple ulcers form throughout the body.

Hande-Schüller-Kisser's disease symptoms Common symptoms Oral mucosal ulcers Eyeballs prominent map-like bone defects Right heart failure Triads Urinary collapse inflammatory cell infiltration

The disease is more common in children over 3 years old, rare in adults, ulcers or xanthoma in the face, eyelids, trunk, perineum and underarm skin, oral mucosal ulcers, hilar and interstitial cells due to tissue cells and inflammatory cells Infiltration and fibrosis can cause right heart failure, pituitary or hypothalamic involvement and diabetes insipidus, skull, skull base, saddle, upper and lower jaw, pelvis, femur, ribs and tibia can be affected, especially limitations Sexual, unequal in size, irregular borders, clear edges, no sclerosing defect area, shaped like a map, so the map-like bone defect, the outer wall of the eyelid and the dome bone are damaged, the soft tissue of the eyelid may be affected to produce eyeball protrusion, but the eyeball is prominent The real cause is unknown. In typical cases, there are skull-like defects, exophthalmos and diabetes insipidus. However, the three characteristics of atypical cases do not occur at the same time, or only one or two of them. Diabetes insipidus is the late stage of the disease. complication.

Inspection of Hande-Schüller-Kisser's disease

Laboratory tests should be performed on atypical lesions, and bone marrow anemia, leukopenia and thrombocytopenia can be found. Lipid giant cells, lymphocytes, eosinophils are found in the bone marrow, and if necessary, biopsy is performed to determine the diagnosis and to determine the presence or absence of bone marrow. Anemia.

Pathological examination: It can be seen that the mature tissue cell nucleus is oval, the nuclear membrane is slightly depressed, the chromatin is thick and dense, the cytoplasmic eosinophilic is obvious, and mononuclear inflammatory cells appear in the background of mature tissue cells, and there are more Eosinophils, scattered in lymphocytes and plasma cells, can also be seen in multinucleated giant cells, tissue cells and multinucleated giant cells phagocytose lipids to lipidate, lesions can be healed by fibrous connective tissue hyperplasia.

1. Cardiac function check: Determine whether there is right heart failure.

2. Imaging examination: X-ray, CT or MRI examination to determine the destruction of the skull, the outer wall of the eyelid and the top wall.

Diagnosis and identification of Hande-Schüller-Kesi

Patients with typical triads, diabetes insipidus, exophthalmos and map-like bone defects, are not difficult to diagnose. Laboratory tests should be performed on atypical lesions, combined with imaging findings to determine the diagnosis.

The lesions that cause bone wall destruction are also myeloma, metastases, etc. The age of myeloma is more than 40 years old. The lesions are small and frequent, and there may be propolis in the urine. The patients with metastatic tumors are usually older and have lesions. Smaller, the edges may not be clear, and the performance of the primary tumor can help diagnose.

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