Xanthoma disseminatum: Difference between revisions
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{{Infobox medical condition | |||
{{Infobox medical condition | |||
| name = Xanthoma disseminatum | | name = Xanthoma disseminatum | ||
| synonyms = Disseminated | | synonyms = [[Disseminated xanthoma]] | ||
| specialty = [[Dermatology]] | |||
| symptoms = Yellowish-brown papules, [[skin lesions]], [[mucous membrane]] involvement | |||
| | | complications = [[Diabetes insipidus]], [[vision impairment]] | ||
| onset = Childhood or early adulthood | |||
| symptoms = | | duration = Chronic | ||
| complications = | | causes = Unknown | ||
| onset = | | risks = Possible association with [[lipid metabolism disorders]] | ||
| duration = | | diagnosis = [[Clinical examination]], [[skin biopsy]] | ||
| differential = [[Xanthomatosis]], [[Erdheim-Chester disease]], [[Langerhans cell histiocytosis]] | |||
| causes = | | treatment = [[Laser therapy]], [[surgical excision]], [[systemic corticosteroids]] | ||
| risks = | | prognosis = Variable, often chronic | ||
| diagnosis = | | frequency = Rare | ||
| differential = | |||
| | |||
| | |||
}} | }} | ||
{{ | {{Short description|A rare skin disorder characterized by the widespread presence of xanthomas}} | ||
'''Xanthoma disseminatum''' is a rare, non-hereditary disorder characterized by the widespread presence of [[xanthomas]], which are yellowish-brown papules and nodules on the skin. This condition is part of a group of disorders known as [[non-Langerhans cell histiocytosis]]. | |||
==Presentation== | |||
[[ | Xanthoma disseminatum typically presents with multiple, symmetrical, yellowish-brown papules and nodules. These lesions are most commonly found on the [[face]], [[neck]], [[trunk]], and [[flexural areas]] such as the [[axillae]] and [[groin]]. The lesions can coalesce to form larger plaques. In some cases, mucosal involvement occurs, affecting areas such as the [[oral cavity]] and [[respiratory tract]]. | ||
==Pathophysiology== | |||
The exact cause of xanthoma disseminatum is unknown. It is characterized by the proliferation of [[histiocytes]], which are a type of [[immune cell]] derived from [[monocytes]]. These histiocytes accumulate in the skin and other tissues, leading to the formation of xanthomas. Unlike other forms of xanthomas, xanthoma disseminatum is not associated with [[hyperlipidemia]]. | |||
==Diagnosis== | |||
The diagnosis of xanthoma disseminatum is primarily clinical, based on the characteristic appearance of the skin lesions. A [[skin biopsy]] can be performed to confirm the diagnosis, revealing the presence of foamy histiocytes and Touton giant cells. [[Immunohistochemistry]] may show positive staining for markers such as [[CD68]] and [[factor XIIIa]]. | |||
==Treatment== | |||
There is no standard treatment for xanthoma disseminatum, and management is often challenging. Options include: | |||
* [[Surgical excision]] of individual lesions | |||
* [[Laser therapy]] | |||
* [[Systemic corticosteroids]] | |||
* [[Chemotherapy]] agents such as [[cyclophosphamide]] | |||
* [[Radiation therapy]] | |||
The response to treatment varies, and spontaneous regression has been reported in some cases. | |||
==Prognosis== | |||
The prognosis of xanthoma disseminatum is variable. While the condition is benign and not life-threatening, it can cause significant cosmetic and functional impairment, especially if mucosal involvement leads to respiratory obstruction. The disease may stabilize or regress over time, but persistent or progressive cases can occur. | |||
==Related pages== | |||
* [[Xanthoma]] | |||
* [[Histiocytosis]] | |||
* [[Non-Langerhans cell histiocytosis]] | |||
[[Category:Dermatology]] | |||
[[Category:Rare diseases]] | |||
[[Category:Skin conditions resulting from errors in metabolism]] | |||
Latest revision as of 05:35, 4 April 2025
| Xanthoma disseminatum | |
|---|---|
| Synonyms | Disseminated xanthoma |
| Pronounce | N/A |
| Specialty | Dermatology |
| Symptoms | Yellowish-brown papules, skin lesions, mucous membrane involvement |
| Complications | Diabetes insipidus, vision impairment |
| Onset | Childhood or early adulthood |
| Duration | Chronic |
| Types | N/A |
| Causes | Unknown |
| Risks | Possible association with lipid metabolism disorders |
| Diagnosis | Clinical examination, skin biopsy |
| Differential diagnosis | Xanthomatosis, Erdheim-Chester disease, Langerhans cell histiocytosis |
| Prevention | N/A |
| Treatment | Laser therapy, surgical excision, systemic corticosteroids |
| Medication | N/A |
| Prognosis | Variable, often chronic |
| Frequency | Rare |
| Deaths | N/A |
A rare skin disorder characterized by the widespread presence of xanthomas
Xanthoma disseminatum is a rare, non-hereditary disorder characterized by the widespread presence of xanthomas, which are yellowish-brown papules and nodules on the skin. This condition is part of a group of disorders known as non-Langerhans cell histiocytosis.
Presentation[edit]
Xanthoma disseminatum typically presents with multiple, symmetrical, yellowish-brown papules and nodules. These lesions are most commonly found on the face, neck, trunk, and flexural areas such as the axillae and groin. The lesions can coalesce to form larger plaques. In some cases, mucosal involvement occurs, affecting areas such as the oral cavity and respiratory tract.
Pathophysiology[edit]
The exact cause of xanthoma disseminatum is unknown. It is characterized by the proliferation of histiocytes, which are a type of immune cell derived from monocytes. These histiocytes accumulate in the skin and other tissues, leading to the formation of xanthomas. Unlike other forms of xanthomas, xanthoma disseminatum is not associated with hyperlipidemia.
Diagnosis[edit]
The diagnosis of xanthoma disseminatum is primarily clinical, based on the characteristic appearance of the skin lesions. A skin biopsy can be performed to confirm the diagnosis, revealing the presence of foamy histiocytes and Touton giant cells. Immunohistochemistry may show positive staining for markers such as CD68 and factor XIIIa.
Treatment[edit]
There is no standard treatment for xanthoma disseminatum, and management is often challenging. Options include:
- Surgical excision of individual lesions
- Laser therapy
- Systemic corticosteroids
- Chemotherapy agents such as cyclophosphamide
- Radiation therapy
The response to treatment varies, and spontaneous regression has been reported in some cases.
Prognosis[edit]
The prognosis of xanthoma disseminatum is variable. While the condition is benign and not life-threatening, it can cause significant cosmetic and functional impairment, especially if mucosal involvement leads to respiratory obstruction. The disease may stabilize or regress over time, but persistent or progressive cases can occur.
