Juvenile xanthogranuloma

Abstract

Juvenile xanthogranuloma (JXG) is a benign, self-healing disorder characterized by solitary or multiple yellow-red nodules on the skin and, occasionally, in other organs. It is predominantly a disease of infancy or early childhood, although adults may also be affected. Histologically, JXG represents an accumulation of histiocytes lacking Birbeck granules (non-Langerhans cells), which can be differentiated from Langerhans cells by specific staining techniques. Affected persons have normal lipid metabolism. JXG is therefore classified as a normolipemic non-Langerhans cell histiocytosis. The patient's general health is not impaired and, in the absence of associated conditions, the prognosis is excellent. Diagnosis is readily made in typical cases, but may be more difficult in unusual variants.

Histiocitose cutânea não-Langerhans como causa de diabetes insípido central

“…O xantogranuloma juvenil é a forma mais comum de histiocitose de células não-Langerhans (13). Acomete principalmente crianças no primeiro ano de vida, podendo, contudo, ocorrer em cerca de 15% dos casos em adultos (2,13).…”

“…Acomete principalmente crianças no primeiro ano de vida, podendo, contudo, ocorrer em cerca de 15% dos casos em adultos (2,13). O quadro clínico clássico é constituído por lesões cutâneas solitárias.…”

Juvenile xanthogranuloma of the nose

“…In addition, malignant tumours such as rhabdomyosarcoma, fibrosarcoma and malignant fibrohistiocytoma should be especially considered when JXG is located in the deep tissues (16).…”

Juvenile xanthogranuloma of the ear: A case report

“…It is the most common form of non-Langerhans cell histiocytosis (2). For the most part, JXG is a benign cutaneous fibrohistiocytic lesion that usually presents during infancy or early childhood.…”

“…Classically, it presents as solitary or multiple yellowish papules or nodules. The most common sites of involvement are the head, neck and trunk (2). In recent years, however, it has become increasingly clear that there are numerous clinical forms of JXG beyond this classic description.…”