What is juvenile xanthogranuloma?
What is juvenile xanthogranuloma?
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 causes JXG?
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.
How is juvenile xanthogranuloma 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.
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.
What is Necrobiotic Xanthogranuloma?
Necrobiotic xanthogranuloma (NXG) is a form of non-Langerhans histiocytosis characterized by the development of red-brown, violaceous, or yellowish cutaneous papules and nodules that evolve to form infiltrated plaques. The periorbital skin is the most common site for NXG.
Is juvenile xanthogranuloma curable?
Juvenile xanthogranuloma is usually self-limiting with skin lesions regressing in 3–6 years. Even systemic JXG resolves spontaneously in most cases.
Can juvenile xanthogranuloma cancerous?
Differential Diagnosis The differential of juvenile xanthogranuloma includes both benign and malignant entities, depending on the clinical presentation and site of involvement.
What does necrobiotic mean?
Necrobiosis is the physiological death of a cell, and can be caused by conditions such as basophilia, erythema, or a tumor. It is identified both with and without necrosis. Necrobiosis.
What causes Paraproteinemia?
Causes. Causes of paraproteinemia include the following: Leukemias and lymphomas of various types, but usually B-cell non-Hodgkin lymphomas with a plasma cell component. Idiopathic (no discernible cause): some of these will be revealed as leukemias or lymphomas over the years.
Is juvenile xanthogranuloma genetic?
Though molecular studies have provided evidence of clonality in juvenile xanthogranuloma, in support of a neoplastic process, little is known about the genetic profile of juvenile xanthogranuloma.
What causes juvenile xanthogranuloma?
Is juvenile xanthogranuloma a tumor?
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 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.
Do Dermatofibromas go away?
Dermatofibromas are harmless growths that develop on the skin. However, they will not usually go away on their own. A person can opt for the surgical removal of unsightly or uncomfortable growths, or they can try a variety of other, less invasive treatments.
Does JXG go away?
Treatment. JXGs on the skin do not usually cause other problems. These usually go away by themselves over several years without any treatment. After the bumps are gone, your child may have a scar or skin color change where the JXG was.
Can dermatofibroma turn cancerous?
Often these start out as red, turning later to brown, and sometimes itch. They probably are a reaction to a minor injury, such a bug bite or a splinter. We do know that they are harmless and never turn cancerous.
What does a dermatofibroma look like?
Cellular dermatofibromas look like small round or oval bumps. They’re usually less than 1 centimeter (cm) in diameter. They may be flesh-colored, brown or reddish-brown. Usually, dermatofibromas form on your arms or legs.
Can juvenile Xanthogranuloma cancerous?
Should dermatofibroma be removed?
Dermatofibromas are generally harmless and don’t usually need to be treated. Although they typically appear in exposed areas like the arms and legs they can occasionally grow elsewhere on the body that is harder to cover up. Sometimes they can also be tender or itchy.
How does dermatofibroma start?
Dermatofibromas are caused by an overgrowth of a mixture of different cell types in the dermis layer of the skin. The reasons why this overgrowth occurs aren’t known. The growths often develop after some type of small trauma to the skin, including a puncture from a splinter or bug bite.
What can be mistaken for dermatofibroma?
Although dermatofibromas are noncancerous growths, sometimes they are mistaken for melanomas. Trauma to a lesion can lead to ulceration and a misdiagnosis of squamous cell carcinoma.
Can dermatofibroma appear suddenly?
There are specific situations where dermatofibromas can be associated with underlying health issues. Typically, multiple dermatofibroma lesions that arise suddenly are seen in these situations. If you have multiple skin lesions that have come on suddenly, you should discuss this with your physician.
Can dermatofibroma be misdiagnosed?
Other variants include aneurysmal, cellular, hemosiderotic, keloidal, granular cell, and lichenoid types of dermatofibroma. Some tumors contain histological or clinical features of several of these variants. This may lead to misdiagnosis in some cases, which may cause difficulty later if the lesion is aggressive.
What do you need to know about juvenile xanthogranuloma?
Juvenile Xanthogranuloma 1 Disease Entity. Juvenile xanthogranuloma (JXG) is a rare benign histiocytic proliferation that develops in infants and young children. 2 Diagnosis. A diagnosis of JXG is suspected in patients with characteristic yellowish cutaneous lesions. 3 Management. 4 Prognosis. 5 References.
How is juvenile xanthogranuloma of the Iris treated?
Early treatment of juvenile xanthogranuloma of the iris with subconjunctival steroids. Br J Ophthalmol 1993;77 (1):57-60. ↑ Cleasby GW.
What is the pathophysiology of Japanese xanthogranuloma?
Pathophysiology. The etiology of JXG is unknown. JXG is believed to result from a disordered macrophage response to a nonspecific tissue injury, resulting in a granulomatous reaction. JXG is on a spectrum of histiocytic disorders that includes benign cephalic histiocytosis, generalized eruptive histiocytosis, adult xanthogranuloma,…
What is the mortality and morbidity of juvenile keratoconjunctivitis (JXG)?
The risk of morbidity is high with ocular involvement and can include hyphema, glaucoma, corneal blood staining, cataract, vascular occlusion, and retinal detachment, all of which can lead to amblyopia in childhood. Rarely, death has been reported among children with visceral JXG.