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. Male child tends to be more affected than female. The disease can affect 10 percent of adults. The exact cause of xanthogranuloma still remains unclear but it is not a hereditary condition. Juvenile xanthogranuloma is characterized by smooth, pink lump in the skin which later on can become yellow or tanned.The lump in skin does not itch or cause pain, but if the surface breaks it can lead to ulceration. Normally the lesion appears on head, neck and trunk of the body. However, in rare cases xanthogranuloma can also develop inside the body in different organs such as the lungs, liver, kidney and heart. Usually in children the disease is self limited and the lesions disappear after few years. In rare instances it may need surgical removal.
The exact cause of xanthogranuloma is still not very well understood by researchers. However, according to some scientists it is believed to occur due to over production of certain type of cells called histiocytes in the body.These cells are a part of body’s immune system. The immune system uses it when there is a nonspecific injury in the body. Histiocytes function is to fight with invading bacteria and dispose of waste products derived from tissue. The disease is rare and it is considered in medical science as non –Langerhans cell histiocytosis. The disease was first reported in 1871 by Rudolf Virchow. In 1954 it was renamed as juvenile xanthogranuloma because of the typical appearance of the cell under the microscope. The disease mainly affects children and infants. In adults the proportion is very less, almost less than 10 percent. Boys are more affected than females.
Juvenile xanthogranuloma usually is a disease of skin. However, it can also affect other parts of the body such as the eye, lungs, liver, kidney, adrenal glands, bones, pituitary gland etc. After skin, eye is the second most common organ to get affected from xanthogranuloma. The lesions of xanthogranuloma are smooth, dome shaped lumps. They are rubbery and firm. The color is usually red or yellow which over a period of time may turn orange. In some cases the lumps are scaly. In majority of cases there is a single lesion. However, multiple lesions are also observed in few cases, especially when there is an associated ocular lesion. Juvenile xanthogranuloma in eye is present in iris and other parts of the eye such as the eyelids. Only one eye is involved. If left untreated, the lesion in eye can lead to blindness however, skin lesion resolve on its own over a period of time spontaneously.
Diagnosis and Treatment:
The diagnosis of xanthogranuloma can be possible from a skin biopsy. Under the microscope, the doctor can observe accumulated and densely packed histiocyte cells. In some cases there may be accompanied fat cells. However, the lipid level in patients suffering from juvenile xanthogranuloma is normal. An ultrasound examination is also performed to know whether the lesions are present in liver, spleen, intestine other organs of abdomen.
Treatment of xanthogranuloma depends on the severity of the condition and the organ involved. The skin lesions are generally self healing and they just need to be observed from time to time. Surgery may be indicated for many reasons such as: for biopsy purpose, or when the function of the affected organ is hampered or due to cosmetic reasons when there are multiple juvenile xanthogranuloma. In case of eye involvement treatment is immediately warranted as there is risk of loss of vision. Treatment for eye lesion is steroid eye drops or steroid is injected into the lesion and taken as pills. In adults, xanthogranuloma usually need treatment while in children the disease is self limited and resolves spontaneously after sometime.