juvenile xanthogranuloma
Introduction
Introduction to juvenile yellow granuloma Juvenile xanthinoma (juvenilexanthogranuloma), also known as juvenile yellow granuloma, formerly known as nevoxxanthoendothelioma or congenital xanthomamultiplex, is a skin, eye and eye caused by benign histiocytosis Orbital lesions, the treatment effect is good, and there is a tendency to self-healing. basic knowledge The proportion of the disease: the disease is rare, the incidence rate is about 0.001% Susceptible people: children Mode of infection: non-infectious Complications: glaucoma
Cause
The cause of juvenile yellow granuloma
(1) Causes of the disease
The cause is currently unclear.
(two) pathogenesis
Its pathogenesis is unknown and may be related to autoimmune diseases.
Prevention
Juvenile yellow granuloma prevention
Maintain good eating habits, pay attention to diet, but not high-fat, high-sugar, high-cholesterol foods.
Complication
Juvenile yellow granuloma complications Complications glaucoma
Granuloma lesions can affect the internal organs such as the gastrointestinal tract, and the corresponding clinical manifestations, repeated bleeding in the eye can lead to secondary glaucoma.
Symptom
Symptoms of juvenile yellow granuloma common symptoms granuloma iris heterochromia nodules bone destruction eyeballs
The disease occurs in infants and children. The skin and eyes are good sites. The internal organs are rarely invaded. The skin of the head, neck and limbs is often invaded by lesions. The skin lesions are usually round, orange, brown or red. Blue nodules, eye and skin nodules may be isolated, or may be part of facial skin nodules, about 1/4 of patients have multiple nodules, generally no more than 10, no ulcers, about 1 /5 patients have skin nodules at birth, disappearing at 2 to 5 years old, lesions can affect the lungs, pericardium, mediastinum, retroperitoneal tissue, striated muscle, gastrointestinal and testis, but generally less common.
Ocular manifestations: Intraocular lesions are generally less prone to occur. The lesions on the surface of the eyeballs such as the conjunctiva, cornea and sclera are much less than those of the orbital lesions. The lesions on the surface of the eye are generally not present at the same time as the lesions in the eye. The age of onset of orbital lesions is larger. The surface of the eyeball and the lesions in the eye are at the same age. In the infant, intraocular lesions often occur in the iris and ciliary body, causing spontaneous anterior chamber hemorrhage. Although the lesion can spontaneously resolve, it has no effect on life, but before it is repeated. House blood, can cause glaucoma and loss of vision, iris lesions may be nodular or diffuse, can also cause iris heterochromia, choroid and retinal involvement is rare, 85% of children with eye disease less than 1 year old, eye disease may be in the skin The disease appeared before.
Eyelid infringement is rare, lesions can invade the humerus and sphenoid bone, causing severe bone destruction. The lesion can be located in the anterior or posterior temporal iliac crest. About 50% of the intraorbital lesions invade the extraocular muscles, and the optic nerve can also be violated. About 1 in 6 patients with skin lesions have eyelid disease, and juvenile yellow granuloma has some association with neurofibromatosis. Milk coffee spots can be found on the patient's trunk skin, and neurofibroma is occasionally found in the eyelids.
Although the eyelid invasion is less, it can invade the bone and cause severe bone destruction. The eye symptoms include eyeball protrusion, periorbital palpable mass and other symptoms and signs of orbital space occupying lesions. The lesion may also involve multiple lesions in the brain. Because of the small lesions, there are no more intracranial symptoms.
Examine
Examination of juvenile yellow granuloma
1. Blood routine examination: Understand the number of blood cells, and if necessary, bone marrow puncture, according to the results, useful for differential diagnosis.
2. Histopathological examination: It is found that the lesions are mainly normal tissue cells, the cells are larger, the nucleus is oval, the cytoplasm is lightly stained, some of the cellular lipids are foamy, and some of the tissue cells are fusiform, in the tissue. The background of the cells shows scattered lymphocytes, plasma cells and a few eosinophils. The prominent histopathological feature of the disease is Touton multinucleated giant cells. The multinuclear ring is located at the center of the cell, and the central region of the nucleus is homogeneous. Eosinophilic cytoplasm, the cytoplasm around the nucleus can be foamy. These Touton multinucleated giant cells are more common in the lesions of the skin and eyelid tissue. There are fewer blood vessels in the skin and orbital lesions, so there is not much chance of bleeding, and the blood vessels in the iris lesion. Many, and located on the surface of the lesion, can be mistaken for iris hemangioma, thin blood vessel wall, so it is easy to cause anterior chamber hemorrhage, electron microscopic examination in the tissue cytoplasm did not see Langerhan particles, old lesions with a large number of fibroblast proliferation Form a scar.
Imaging examination: Ultrasound, CT and MRI are very similar to Langerhans cell histiocytosis, which is characterized by soft tissue block of the eyelid, multiple bone destruction of the humerus and intracranial, MRI shows sputum, multiple lesions of the skull, such as bone destruction X-ray can show multiple lesions of intracranial bone destruction, with clear boundaries but not neat.
Diagnosis
Diagnosis and diagnosis of juvenile yellow granuloma
diagnosis
Infants and children have high facial and neck skin, orange or purple-red nodules, spontaneous bleeding in the anterior chamber or eyelid involvement. These are typical clinical manifestations of juvenile yellow granuloma. It is best to remove skin nodules. Histopathological examination reveals that a large number of tissue cells and scattered Touton multinucleated giant cells can be found in the lesion to establish a diagnosis.
Differential diagnosis
However, it should be distinguished from histiocytosis X. The visceral system of patients is rarely affected by Letterer-Siwe disease; no map-like skull destruction and diabetes insipidus can be distinguished from Hand-Schüller-Christian disease; there is no Langerhans granule in tissue cytoplasm. It can be distinguished from eosinophilic granuloma; the patient's cervical lymph nodes are not enlarged, and are not the same as sinus augmentation.
