Geographic bone defect

Introduction A map-like bone defect is one of the symptoms of Hand-Schüller-Kisser's disease. The typical triad of patients with Hande-Schüller-Kersi disease is diabetes insipidus, exophthalmos and map-like bone defects, and the diagnosis is not difficult. The disease is more common in children over 3 years old, and adults are rare. The patient's face, eyelids, trunk, perineum and underarm skin have ulcers or yellow tumors, and oral mucosal ulcers. The hilar and pulmonary interstitial fibrosis due to infiltration of tissue cells and inflammatory cells can cause right heart failure.

Cause

(1) Causes of the disease

The cause is unknown and may be related to immune dysfunction.

(two) pathogenesis

The pathogenesis is unclear.

an examination

Related inspection

Bone imaging and sacral area CT examination

symptom:

The disease is more common in children over 3 years old, and adults are rare. The patient's face, eyelids, trunk, perineum and underarm skin have ulcers or yellow tumors, and oral mucosal ulcers. The hilar and pulmonary interstitial fibrosis due to infiltration of tissue cells and inflammatory cells can cause right heart failure. Diabetes insipidus occurs when the pituitary or hypothalamus is involved. The skull, skull base, saddle, upper and lower jaw, pelvis, femur, ribs and tibia can be involved, especially in areas of limitation, size, irregular boundaries, clear edges, and no hardening, shaped like a map. Therefore, it is called a map-like bone defect. The outer wall of the eyelid and the parietal bone are damaged, and the soft tissue of the eyelid may be affected to produce eyeball protrusion, but the real cause of the eyeball is unknown. In typical cases, there are three types of skull map-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 a late complication of the disease.

Diagnosis :

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.

Differential diagnosis

The lesions that cause bone wall destruction are also myeloma and metastases. The age of myeloma is more than 40 years old, the lesions are small and multiple, there may be condensed protein in the urine; patients with metastatic tumors are usually older, the lesions are smaller, the edges may not be clear, the performance of primary tumors can help diagnose .

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