What is JXG disease?
A juvenile xanthogranuloma (zan tho grahn you LOH mah), or JXG, is a benign skin lump or bump caused by a collection of cells called histiocytes. These may be red, orange or tan at first, but over time may become more yellow in color. These bumps usually appear on the head, neck and trunk.
How is JXG treated?
The first-line treatment of iris JXG is high-dose corticosteroids applied topically (every two hours during waking hours and ointment at night) with a slow taper. If there is difficulty administering topical medication, periocular steroids can be considered, although the effects of the steroids may last for 3-4 months.
What is a Xanthogranuloma?
Xanthogranulomas are benign, usually asymptomatic, self-healing, red, yellow, or brown papules, nodules composed of histiocytic cells that predominantly occur in infancy and childhood. Papules, nodules occur in skin, eyes, and viscera. Adamson first reported juvenile xanthogranuloma (JXG) in 1905.
How common is juvenile xanthogranuloma?
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.
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 malignant?
Juvenile xanthogranuloma (JXG) is a benign, proliferative disorder of histiocytic cells of the dermal dendrocyte phenotype. JXG belongs to the broad group of non-Langerhans cell histiocytoses and is typically a disorder of early childhood.
What is Xanthogranuloma adult?
Xanthogranuloma is a rare benign tumour, part of the non-Langerhans cell histiocytosis group, uncommon in adults and even less common in the paranasal sinuses. Despite its benign nature, it mimics neoplasm due to its local effects which can have serious functional consequences depending on the anatomical location.
What does a histiocyte do?
A histiocyte is a type of immune cell. It destroys foreign substances to protect the body from infection.
What causes histiocytic inflammation?
The exact cause of histiocytosis is unknown. However, recent studies indicate that it is caused by the development and expansion of an abnormal Langerhans cell that subsequently leads to the accumulation of other cells of the immune system, resulting in collections or tumors in various areas of the body.
Where are histiocytes found?
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.
What does histiocytic infiltrate mean?
Abstract. Histiocytic disorders (HDs) are a diverse group of diseases characterized by pathologic infiltration of normal tissues by cells of the mononuclear phagocyte system. The spectrum of these diseases ranges from treatable infectious diseases to rapidly progressive, life-threatening conditions.
What do histiocytes look like?
Histiocytes (Macrophages) In smears, the typical histiocyte is easily identified by its size, eccentric round or bean-shaped nucleus, and lightly stained lacy cytoplasm (Fig. 19.43). Because histiocytes are phagocytic, their cytoplasm may contain leukocytes, nuclear particles (Fig.
Are histiocytes cancerous?
Langerhans cell histiocytosis is a rare disorder that can damage tissue or cause lesions to form in one or more places in the body. It is not known whether LCH is a form of cancer or a cancer-like disease.
What does histiocytic mean?
Listen to pronunciation. (HIS-tee-oh-sy-TOH-sis) A group of rare disorders in which too many histiocytes (a type of white blood cell) build up in certain tissues and organs, including the skin, bones, spleen, liver, lungs, and lymph nodes.
Are histiocytes normal?
Does histiocytosis go away?
Not all patients with Langerhans cell histiocytosis need treatment. If the disease is limited to the skin or one bone lesion, it likely will go away on its own.
What causes histiocytosis?
Is histiocytosis genetic?
Langerhans cell histiocytosis is a rare disorder that can affect people of all ages. The highest rate is among children ages 5 to 10. Some forms of the disorder are genetic, which means they are inherited.