Xanthoma disseminatum: Difference between revisions

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{{Infobox medical condition (new)
{{Short description|A rare skin disorder characterized by the widespread presence of xanthomas}}
| name            = Xanthoma disseminatum
| synonyms        = Disseminated xanthosiderohistiocytosis<ref name="Bolognia">{{cite book |author=Rapini, Ronald P. |author2=Bolognia, Jean L. |author3=Jorizzo, Joseph L. |title=Dermatology: 2-Volume Set |publisher=Mosby |location=St. Louis |year=2007 |pages= |isbn=978-1-4160-2999-1 |oclc= |doi= |accessdate=}}</ref> and Montgomery syndrome<ref name="Andrews" />
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'''Xanthoma disseminatum''' is a rare cutaneous condition that preferentially affects males in childhood, characterized by the insidious onset of small, yellow-red to brown [[papule]]s and nodules that are discrete and disseminated.<ref name="Andrews">{{cite book |author=James, William D. |author2=Berger, Timothy G.|title=Andrews' Diseases of the Skin: clinical Dermatology |publisher=Saunders Elsevier |location= |year=2006 |pages= |isbn=978-0-7216-2921-6 |oclc= |doi= |accessdate=|display-authors=etal}}</ref>{{rp|717}}


It is a [[histiocytosis]] syndrome.<ref name="pmid15681329">{{cite journal |vauthors=Alexander AS, Turner R, Uniate L, Pearcy RG |title=Xanthoma disseminatum: a case report and literature review |journal=Br J Radiol |volume=78 |issue=926 |pages=153–7 |date=February 2005 |pmid=15681329 |doi= 10.1259/bjr/27500851|url=}}</ref>
'''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]].


== See also ==
==Presentation==
* [[Non-X histiocytosis]]
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]].
* [[List of cutaneous conditions]]


== References ==
==Pathophysiology==
{{reflist}}
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]].
== External links ==
{{Medical resources
|  DiseasesDB    = 
|  ICD10          = E78.2 ([[ILDS]] E78.240)
|  ICD9          = 
|  ICDO          = 
|  OMIM          = 
|  MedlinePlus    = 
|  eMedicineSubj  = 
|  eMedicineTopic = 
|  MeshID        =
}}
{{Lipid metabolism disorders}}
{{Histiocytosis}}


[[Category:Monocyte- and macrophage-related cutaneous conditions]]
==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]]


{{Cutaneous-condition-stub}}
The response to treatment varies, and spontaneous regression has been reported in some cases.
{{stb}}
 
{{No image}}
==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]]

Revision as of 19:07, 22 March 2025

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:

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

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