How is Xanthogranuloma treated?
Pharmacotherapy: Topical, subconjunctival, intralesional, and systemic corticosteroids are used for intraocular and orbital juvenile xanthogranuloma (JXG). Orbital lesions may respond to intralesional steroid injections. Iris lesions are treated with topical prednisolone or subconjunctival steroids.
What causes Xanthogranuloma?
The cause of JXG is not clearly understood but it is believed to result from an overproduction of a type of histiocyte cell used in the body’s immune system in response to nonspecific tissue injury. Histiocytes work in the immune system to fight bacteria and dispose of tissue waste products.
What does Xanthogranuloma mean?
Xanthogranulomas are nodules in the skin of neonates, infants, and adults that are characterized histologically by large foamy macrophages (xanthoma cells). Their natural history and age distribution suggest that they are not neoplasms but reactive developmental/ metabolic or inflammatory lesions.
How long does it take for JXG to go away?
JXG can sometimes be seen at birth. Other times the bumps can form after birth for up to 1 to 2 years. The bumps normally go away on their own in children over 3 to 6 years.
Is Xanthogranuloma benign?
Xanthogranulomas are benign, usually asymptomatic, self-healing, red, yellow, or brown papules, nodules composed of histiocytic cells that predominantly occur in infancy and childhood.
What is benign cephalic histiocytosis?
Benign cephalic histiocytosis (BCH) is a rare type of non-Langerhans histiocytitic disorder and is characterized by self-healing multiple small eruptions of yellow to red-brown papules on the face and upper trunk.
What does a histiocyte do?
A histiocyte is a normal immune cell that is found in many parts of the body especially in the bone marrow, the blood stream, the skin, the liver, the lungs, the lymph glands and the spleen. In histiocytosis, the histiocytes move into tissues where they are not normally found and cause damage to those tissues.
How common is JXG?
JXG is considered a rare disease in itself, but is the most common type of non-Langerhans histiocytosis. The incidence of juvenile xanthogranuloma is estimated to be 1 per million in children, however it is probably underdiagnosed. Up to 10% of patients with neurofibromatosis type I may develop JXG.
What is the best treatment for xanthogranuloma of the orbit?
Adult xanthogranuloma of the orbit is rare, making prospective evaluation or meta-analysis impossible. The best treatment is unknown but seems to be with multiagent chemotherapy guided by histopathological, immunohistochemical, and systemic findings.
How is xanthogranuloma treated in erdheim-chester disease?
Xanthogranuloma presents with fibrosclerosis of the orbital tissues. In Erdheim-Chester disease, the mediastinum, pericardium, pleura, bone, and retroperitoneum may be involved, and orbital disease is more diffuse with potential for vision loss. Local lesions are treated with systemic corticosteroids and other immunosuppressants.
What are the causes and symptoms of xanthogranuloma?
Causes, Symptoms and Treatment 1 Causes: The exact cause of xanthogranuloma is still not very well understood by researchers. 2 Symptoms: Juvenile xanthogranuloma usually is a disease of skin. 3 Diagnosis and Treatment: The diagnosis of xanthogranuloma can be possible from a skin biopsy.
What is juvenile xanthogranuloma?
Juvenile xanthogranuloma is a disease mainly affecting skin. It is a disease in which there is an excess of histiocytes, a type of cells in the body. This disease is a rare and it predominantly occurs in infants and children.