According to aviationopedia.com, Juvenile xanthogranuloma mostly affects children under the age of one and often regresses or fades on its own. It is a yellow-orange patch or benign hemispherical tumor. As long as it is not localized on the eye, the xanthogranuloma only needs medical treatment in very rare cases.
What is juvenile xanthogranuloma?
As a rule, a small lump is visible in the child. However, if the disease is directly on the eye or in the vicinity of the eye, an ophthalmologist must be consulted.
A juvenile xanthogranuloma (JXG) is a benign non-Langerhans cell histiocytosis that occurs predominantly in infants and young children under the age of two. It is a collection of lipid-laden macrophages. Smooth, dome-shaped nodules and papules with the typical yellow-orange coloring can appear at birth.
It most commonly occurs around age 2. Rare in older children and even rarer in adults. Benign means benign and non-aggressive. In individual cases, other organs may be involved. These include the eyes, spleen, liver, lungs and central nervous system, but this is comparatively rare. Juvenile xanthogranuloma is also known as non-X histiocytosis, juvenile xanthogranuloma or naevoxanthoendothelioma.
Causes
Nothing precise is known about the causes of xanthogranulomas. It is possible that genetic dispositions are behind the development of these skin changes. In any case, this is an overproduction of histiocytes and dendritic cells, which accumulate and lead to different symptoms depending on the location.
Symptoms, Ailments & Signs
JXG are usually solitary, i.e. individually occurring yellow-orange nodules or raised papules with a diameter of 0.5 to 2 centimeters. These can also appear reddish in the beginning. They are mainly visible in the head area, on the neck or upper body and on the extensor sides of the extremities and are relatively harmless.
However, if they occur in or near the eyes and possibly even multiple times, an ophthalmologist should be consulted. An ophthalmological examination must be carried out to clarify whether there is a risk to the eyesight. In rare cases, the juvenile xanthogranuloma can establish itself in other organs. There are a whole range of options for this. In addition to the above, they have also been observed in the kidneys, bones, muscle tissue, adrenal glands, testes (testicles), larynx (windpipe), intestines and pericardium (heart muscle).
Diagnosis & course of disease
A visual diagnosis is often sufficient to recognize the characteristic skin signs. Modern examination devices and high-resolution microscopes enable the little patients to carry out more gentle diagnostic procedures than just a few years ago. Infants affected from birth and up to the first six months of life are more likely to have multiple lesions.
A case frequency of ten percent of births is assumed and it is noteworthy that male infants are affected more frequently. In unclear cases, Langerhans histiocytosis, neurofibromatosis type 1, and juvenile myelomonocyte leukemia must be ruled out. These are the most common dangerous conditions that JXG can be confused with.
Other differential diagnoses are: molluscum contagiosum, Feuerstein-Mims-Schimmelpenning syndrome, scar keloid, urticaria pigmentosa, histiocytoma, fibromatosis, digital, infantile, Spitz nevus, fibroma, xanthoma tuberosum and eruptive. Normally, tissue samples are not taken from infants and young children unless it is absolutely necessary, because the juvenile xanthogranuloma usually disappears on its own by the age of six.
The most common site of extracutaneous occurrence is the eyes with the eyelid and iris. About half of these small patients have skin lesions and thus juvenile xanthogranulomatosis is the most common cause of spontaneous hyphema in the iris and ciliary body.
This means an accumulation of red blood cells in the front of the eye, as occurs with blunt trauma, for example in boxing. In the children affected, this leads to secondary glaucoma and, as a further consequence, often even to the point of blindness. The treatment is therefore a great challenge for every ophthalmologist.
If the disease occurs in adults, the diagnosis is difficult and a high number of unreported cases is assumed. In addition, there is no longer any spontaneous healing, as is the case with most children. Depending on the location, a wide variety of symptoms occur, which can also be assigned to numerous other diseases. Therefore, these diagnoses may be incidental findings in the context of diagnosing other diseases.
Complications
In most cases, this condition does not require any special treatment. In most cases, the spot or benign tumor will go away on its own, with no further complications or discomfort. As a rule, a small lump is visible in the child. However, if the disease is directly on the eye or in the vicinity of the eye, an ophthalmologist must be consulted.
In this case, the lump can affect the affected person’s vision and thus reduce the patient’s quality of life. The disease can also occur in other internal organs and lead to symptoms or complications there as well. The further course mostly depends on the affected region of the patient. In most cases, treatment of this disease is not necessary.
The symptoms usually go away on their own without causing any particular complications. This does not affect the life expectancy of the patient. The tumor can also be limited by radiation or chemotherapy. Usually there are no further complications. In some cases, psychological support for the parents or relatives is also necessary.
When should you go to the doctor?
Normally, a doctor’s visit is not necessary for a juvenile xanthogranuloma. The skin abnormalities appear in newborns and infants. Spontaneous healing occurs within a few weeks or months without any further sequelae. A visit to the doctor can be arranged to reassure and reassure the parents. Often, however, the information from the obstetrician is already sufficient through visual contact. Since there are routine check-ups of the newborn within the first few months of life, the skin changes are usually addressed at these treatment appointments and clarified by a doctor.
Rarely, the granuloma develops in areas of the body that require medical intervention. If the changes in the skin’s appearance develop in the vicinity of the eyes, vision impairments can occur. If discoloration of the skin or new tissue formation in the form of poplars is observed in the region around the eyelids, a doctor should be consulted. If a risk to vision is identified, medical treatment is advised. In exceptional cases, the granulomas can attach themselves to various organs inside the body.
A doctor should therefore be consulted if there are any abnormalities in digestion or a decrease in the infant’s appetite. If you lose a lot of weight, if you experience diarrhea, constipation or pain in your stomach or kidneys, you should see a doctor.
Treatment & Therapy
There is no special therapy for babies and small children, and it is usually not appropriate either, since juvenile xanthogranulomas often disappear on their own. However, if they are close to the eyes, very low doses of radiation can be used to curb growth.
There are various options for extracutaneous JXG, depending on the location. For larger accumulations in the abdomen and organs and nervous system, chemotherapy similar to therapy for histiocytosis X can be used. Extracutaneous JXG is one of the rare diseases for which hardly any research funds are made available. Therefore, there is no standard therapy that can be used in individual cases.
Outlook & Forecast
In almost all patients, the juvenile xanthogranuloma has a favorable prognosis. In most cases, spontaneous healing occurs. The changes in the appearance of the skin do not pose any further threat to the health of the person concerned. Treatment or the intervention of a doctor is only rarely necessary. The granulomas appear primarily in children and disappear within a few days, weeks or months.
If the skin changes have not been scratched and open wounds have developed, they have no disease value. If there are open wounds, there is a risk of blood poisoning. The wounds can become contaminated and germs can get into the organism via the entry points. A worsening of the prognosis is to be expected, since sepsis is potentially life-threatening. In addition, complications can arise.
If granulomas develop near the eye, the otherwise favorable prognosis also deteriorates. In this part of the body, they can lead to impairment of vision. Medical care is necessary to avoid complications and to remove the skin changes. If the treatment proceeds without further incidents, the patient can also be discharged from the treatment within a short time as recovered. A recurrence is possible in the further course, especially during childhood. The prognosis is favorable in these cases.
Prevention
There is no way to prevent juvenile xanthogranuloma and the mechanisms by which it occurs have not yet been fully clarified. The Histiocytosis Association is working closely with an international group of doctors to advance research in this area and give these rare patients a chance for a future cure.
Aftercare
In many cases, there are no special follow-up measures available to those affected by this disease. First and foremost, a very quick diagnosis with subsequent treatment is important so that there are no further complications in the life of the person affected. In some cases, this tumor can go away on its own, but the disease should still be checked out by a doctor.
If the disease goes away on its own, no special follow-up care is necessary or possible. However, those affected should still regularly undergo various examinations and check-ups by a doctor in order to identify and treat further tumors or other complaints at an early stage. Since the disease primarily occurs in children, they need to be adequately supported by their parents and other family members.
Intensive and loving conversations are also very important here, as this primarily prevents depression and other mental upsets. Parents should also be careful that children do not scratch the lesions on the skin. Only in a few cases does this disease limit the life expectancy of those affected.
You can do that yourself
For patients with a juvenile xanthogranuloma, it is of the utmost importance to carefully monitor the changes associated with the disease and to visit the treating doctor immediately in the event of enlargements or other innovations in the granuloma. It is also recommended that the parents take the affected child to regular preventive check-ups in order to register changes in good time and initiate appropriate therapy.
In many cases, the juvenile xanthogranuloma disappears on its own in the young affected and does not require any medical treatment. Then patients and guardians only support the course of the disease by being careful not to irritate the juvenile xanthogranuloma. Irritations are possible, for example, from cosmetics or injuries caused by rubbing clothing or handles during personal hygiene. Therefore, during such activities, patients are especially attentive and careful not to provoke negative changes.
Treatment is necessary when the granuloma is close to the eye and threatens to impair visual function. In such cases, care by ophthalmologists and other specialists is required to remove the juvenile xanthogranuloma and thus prevent complications in good time.
