Association of solitary, segmental hemangiomas of the skin with visceral hemangiomatosis.

Association of solitary, segmental hemangiomas of the skin with visceral hemangiomatosis. Research Abstract Details 

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Association of solitary, segmental hemangiomas of the skin with visceral hemangiomatosis. Abstract Text:

BACKGROUND: Multiple hemangiomas of the skin have traditionally been recognized as a clue to potential visceral hemangiomas. Recently, hemangiomas have been recognized to have subcategories, localized and segmental, which correlate with risk of complications. While less common, segmental hemangiomas of the skin have a higher risk of being life- or function-threatening and/or having associated structural anomalies such as those that occur in PHACE (posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities) syndrome (PHACES, if sternal clefting/supraumbilical raphe is included). However, the potential association of solitary, segmental hemangiomas of the skin with visceral hemangiomatosis has not been previously emphasized. OBSERVATIONS: A total of 47 cases of segmental hemangiomas of the skin in association with visceral hemangiomatosis were found. The location of the cutaneous hemangiomas most commonly, but not exclusively, involved the face (37 cases [79%]). The most common site of internal organ involvement was the liver (20 cases [43%]), followed by the gastrointestinal tract (16 [34%]), brain (16 [34%]), mediastinum (9 [19%]), and lung (7 [15%]). The percentages of reported cases of hemangiomas of the pancreas, spleen, bones, or kidneys were 6% or less. Forty percent of patients met criteria for the diagnosis of PHACE(S) syndrome. In this subgroup, internal organ hemangiomas were most commonly found in the brain or mediastinum (18 cases [53%]). Overall, 12 patients (25%) died during infancy, most commonly because of gastrointestinal involvement or congestive heart failure secondary to liver involvement.Conclusion Segmental hemangiomas of the skin have an associated risk of visceral hemangiomatosis, with the potential of causing vital organ compromise.

Association of solitary, segmental hemangiomas of the skin with visceral hemangiomatosis. Publishing Authors By Initials

For similar neoplasms: neoplasms by site: head and neck neoplasms: tracheal neoplasms research abstracts see: neoplasms: neoplasms by site: head and neck neoplasms: tracheal neoplasms research

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Association of solitary, segmental hemangiomas of the skin with visceral hemangiomatosis. Journal Published:

PUBLICATION TYPE: Review

Journal: Archives of dermatology

VOLUME: 140

Page Numbers: 591-6

Journal Abbreviation: Arch Dermatol

ISSN: 0003-987X

DAY: 17

MONTH: May

YEAR: 2004

Association of solitary, segmental hemangiomas of the skin with visceral hemangiomatosis. Information

Number of References: 32

LANGUAGE: eng

NlmUniqueID: 372433

Association of solitary, segmental hemangiomas of the skin with visceral hemangiomatosis. Keywords Mesh Terms:

KEYWORDS: Tracheal Neoplasms

MESH TERMS: radiography

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Grant and Affiliation Information for Association of solitary, segmental hemangiomas of the skin with visceral hemangiomatosis.

AFFILIATION: Department of Dermatology, Baylor College of Medicine, Houston, Texas 77030, USA. dmetry@bcm.tmc.edu

Country: United States

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MEDLINETA: Arch Dermatol

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