What is xanthoma disseminatum?

What is xanthoma disseminatum?

Xanthoma disseminatum, also known as Montgomery syndrome, is a rare non-Langerhans cell-derived benign histiocytosis disease with unknown etiology. The disease occurs most frequently in men, ranging in age from 8 months to 85 years, but 60% of patients develop the disease before the age of 25 years [2].

What causes xanthoma disseminatum?

The cause of xanthoma disseminatum is unknown, and treatment generally is unsatisfactory. Upper respiratory involvement has been reported most commonly in the buccal mucosa, larynx, and pharynx, but few reports describe in detail involvement of the lower respiratory tract.

What is the difference between xanthoma and xanthelasma?

Xanthomas may appear anywhere on the body. The most common places are the elbows, joints, tendons, knees, hands, feet, and buttocks. If the fatty lumps are on the eyelids, it’s called xanthelasma.

What causes xanthoma?

Xanthoma is usually caused by high levels of blood lipids, or fats. This may be a symptom of an underlying medical condition, such as: hyperlipidemia, or high blood cholesterol levels. diabetes, a group of diseases that causes high blood sugar levels.

Are xanthomas common?

Xanthomas are common, especially among older adults and people with high blood lipids (fats). Xanthomas vary in size. Some are very small.

Will xanthomas go away?

‌In some cases, once you get your blood lipid levels down, the xanthomas will go away on their own. If not, you can speak to your doctor about having them removed.

How rare is JXG?

In 40–70% of patients, JXG develops in the first year of life [5]. In 5–17% of cases, the skin lesions may appear soon after birth [3, 5]. The highest incidence of JXG in adulthood is observed in patients aged 20 to 30 years, but the disease is generally rare in adults.

What is xanthoma disseminatum (XD)?

Xanthoma disseminatum (XD) is a rare, normolipemic form of histiocytosis. Patients rapidly develop hundreds of red-brown papules that become increasingly yellow with time. The lesions are symmetrically distributed and involve the eyelids, trunk, face, and flexural folds of the proximal extremities.

What is the pathophysiology of xanthomas?

Xanthomas are usually a skin sign of disorders of lipid metabolism (dyslipidaemias) or occur in histiocytosis; the former is the focus of this page. Xanthomas typically affect adults, although children with familial hypercholesterolaemia may present with xanthomas.

Which histologic findings are characteristic of xanthogranuloma disseminatum (XRD)?

Skin biopsy specimens of xanthogranuloma disseminatum will demonstrate a mixture of histiocytes, foam cells, and inflammatory cells. If a later lesion is biopsied a predominance of foam cells may be observed, as well as Touton giant cells. Immunohistochemical staining will demonstrate S100 negativity, CD1a negativity, and CD68 positivity.

What is xanthelasma palpebrarum?

Xanthelasma (also known as xanthelasma palpebrarum or xanthelasma palpebrum), is the most common form of xanthoma. It is a type of plane xanthoma usually located around the medial canthus of the upper eyelid. Upper and lower eyelids can be affected symmetrically.