Disseminated xanthoma with diabetes insipidus
Introduction
Introduction to disseminated xanthoma with diabetes insipidus Disseminated xanthoma with diabetes insipidus (xanthomadisseminatum withdiabetesinsipidus) is a skin mucosal disseminated xanthoma with diabetes insipidus, a specific combination of the two. Rarely, mainly in young children and adolescents, the ratio of male to female is 2:1, and the genetic pattern is unknown. basic knowledge The proportion of sickness: 0.00001% Susceptible population: occurs in young children and adolescents Mode of infection: non-infectious complication:
Cause
Disseminated xanthoma with the cause of diabetes insipidus
(1) Causes of the disease
The disease is a normal blood lipid xanthomatosis, occasionally secondary hyperlipidemia (increased cholesterol levels), about 50% of cases with diabetes insipidus.
(two) pathogenesis
The occurrence of diabetes insipidus may be caused by nodular xanthomas on the dura mater, which is caused by compression of the pituitary pedicle or hypothalamus. Some scholars attribute the disease to the histiocytosis group, but there is no tissue cell proliferation. The Langerhans particles were not found in the tissue-like cells, and the S-100 and OKT6 antibodies were negatively stained. It is interesting to note that the new papules showed a single form of histiocytosis, followed by a polymorphous inflammatory granuloma. A large number of lipid-containing and xanthomatic cells and lipoprotein-containing Totun giant cells.
Prevention
Disseminated xanthoma with diabetes insipidus prevention
The disease is a normal hyperlipidemia, occasionally secondary hyperlipidemia (increased cholesterol levels), about 50% of cases with diabetes insipidus.
Complication
Disseminated xanthoma with complications of diabetes insipidus Complication
Generally no special complications
Symptom
Disseminated xanthoma with symptoms of diabetes insipidus common symptoms nodular diuretic pimples
There are three major characteristic symptoms in clinical practice:
1. Disseminated skin papular xanthomas lesions are hemispherical papules and nodular, dark yellow or brownish yellow, which can grow to pea size or larger, the main symmetry is distributed in the eyes, both sides of the neck And the lateral side of the joint, the lesion can be fused into a braid, turning red or brown.
2. Mucosal xanthoma lesions occur frequently, almost every case can occur, mainly in the oral cavity, posterior pharyngeal wall, nasopharynx and throat, can also be seen in the bronchi or even bronchioles, xanthoma can even occur in the binding membrane and tonsil There are also reports of brain xanthoma.
3. About 50% of patients with diabetes insipidus have diabetes insipidus, partly because of the response to diuretic hormones, and the urine specific gravity is sometimes relatively high. This is not common.
Lipoprotein metabolism is normal, triglyceride and cholesterol levels tend to be normal, but the disease process may also increase, the cause is unclear, bone marrow, brain, respiratory tract, heart, kidney, liver, pancreas, lymph nodes, uterus and muscles, etc. Organs can be affected.
Examine
Disseminated xanthoma with examination of diabetes insipidus
Histopathology: The initial lesion is a single form of histiocytosis, which subsequently develops into an inflammatory granuloma with a large number of white blood cells, eosinophils, lymphocytes and macrophages infiltrating, and finally forms lipid-rich and lipoprotein-rich. Large and small xanthoma cells, Touton-type giant cells.
Diagnosis
Diagnosis and differentiation of disseminated xanthoma with diabetes insipidus
According to the clinical manifestations, the characteristics of skin lesions and histopathological features can be diagnosed. Other types of nodular xanthoma must be ruled out, such as juvenile yellow granuloma, papular xanthomas, and histiocytosis X with xanthoid lesions.
