Angiomatosis

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Angiomatosis
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Skin angiomatosis
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Angiomatosis is a non-neoplastic condition [1] characterised by nests of proliferating capillaries arranged in a lobular pattern, displacing adjacent muscle and fat. [2] It consists of many angiomas. [3]

Contents

These tend to be cavernous hemangiomas, which are sharply defined, sponge-like tumors composed of large, dilated, cavernous vascular spaces.[ citation needed ]

Presentation

Associated

They often appear in:

Histology

It is a vascular malformation wherein blood vessels proliferate along with accompanying mature fat and fibrous tissue, lymphatics and sometimes nerves. [2] They may involve skin, subcutaneous tissue, skeletal muscle and occasionally bone. [2]

Prognosis

Prognosis depends on the size and location of the tumour, untreated angiomatosis may lead to blindness and/ or permanent brain damage. Death may occur, with complications in the kidney or brain. [4]

See also

Related Research Articles

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<span class="mw-page-title-main">Infantile hemangioma</span> Raised red skin lesion that affects infants caused by benign vascular tumor

An infantile hemangioma (IH), sometimes called a strawberry mark due to appearance, is a type of benign vascular tumor or anomaly that affects babies. Other names include capillary hemangioma, "strawberry hemangioma", strawberry birthmark and strawberry nevus. and formerly known as a cavernous hemangioma. They appear as a red or blue raised lesion on the skin. Typically, they begin during the first four weeks of life, growing until about five months of life, and then shrinking in size and disappearing over the next few years. Often skin changes remain after they shrink. Complications may include pain, bleeding, ulcer formation, disfigurement, or heart failure. It is the most common tumor of orbit and periorbital areas in childhood. It may occur in the skin, subcutaneous tissues and mucous membranes of oral cavities and lips as well as in extracutaneous locations including the liver and gastrointestinal tract.

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<span class="mw-page-title-main">Sturge–Weber syndrome</span> Medical condition

Sturge–Weber syndrome, sometimes referred to as encephalotrigeminal angiomatosis, is a rare congenital neurological and skin disorder. It is one of the phakomatoses and is often associated with port-wine stains of the face, glaucoma, seizures, intellectual disability, and ipsilateral leptomeningeal angioma. Sturge–Weber syndrome can be classified into three different types. Type 1 includes facial and leptomeningeal angiomas as well as the possibility of glaucoma or choroidal lesions. Normally, only one side of the brain is affected. This type is the most common. Type 2 involvement includes a facial angioma with a possibility of glaucoma developing. There is no evidence of brain involvement. Symptoms can show at any time beyond the initial diagnosis of the facial angioma. The symptoms can include glaucoma, cerebral blood flow abnormalities and headaches. More research is needed on this type of Sturge–Weber syndrome. Type 3 has leptomeningeal angioma involvement exclusively. The facial angioma is absent and glaucoma rarely occurs. This type is only diagnosed via brain scan.

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<span class="mw-page-title-main">Vascular tumor</span> Medical condition

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<span class="mw-page-title-main">Vascular disease</span> Medical condition

Vascular disease is a class of diseases of the vessels of the circulatory system in the body, including blood vessels – the arteries and veins, and the lymphatic vessels. Vascular disease is a subgroup of cardiovascular disease. Disorders in this vast network of blood and lymph vessels can cause a range of health problems that can sometimes become severe, and fatal. Coronary heart disease for example, is the leading cause of death for men and women in the United States.

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<span class="mw-page-title-main">Bonnet–Dechaume–Blanc syndrome</span> Medical condition

Bonnet–Dechaume–Blanc syndrome, also known as Wyburn-Mason syndrome, is a rare congenital disorder characterized by arteriovenous malformations of the brain, retina or facial nevi. The syndrome has a number of possible symptoms and can, more rarely, affect the skin, bones, kidneys, muscles, and gastrointestinal tract. When the syndrome affects the brain, people can experience severe headaches, seizures, acute stroke, meningism, and progressive neurological deficits due to acute or chronic ischaemia caused by arteriovenous shunting.

<span class="mw-page-title-main">Blue rubber bleb nevus syndrome</span> Medical condition

Blue rubber bleb nevus syndrome is a rare disorder that consists mainly of abnormal blood vessels affecting the skin or internal organs – usually the gastrointestinal tract. The disease is characterized by the presence of fluid-filled blisters (blebs) as visible, circumscribed, chronic lesions (nevi).

A vascular anomaly is any of a range of lesions from a simple birthmark to a large tumor that may be disfiguring. They are caused by a disorder of the vascular system. A vascular anomaly is a localized defect in blood or lymph vessels. These defects are characterized by an increased number of vessels, and vessels that are both enlarged and sinuous. Some vascular anomalies are congenital, others appear within weeks to years after birth, and others are acquired by trauma or during pregnancy. Inherited vascular anomalies are also described and often present with a number of lesions that increase with age. Vascular anomalies can also be a part of a syndrome.

PHACE syndrome is a medical condition involving the uncommon association between large infantile hemangiomas, usually of the face, and birth defects of the brain, heart, eyes, skin and/or arteries. PHACE is an acronym that stands for the medical names of the parts of the body it often impacts:

<span class="mw-page-title-main">Cavernous hemangioma</span> Human disease

Cavernous hemangioma, also called cavernous angioma, venous malformation, or cavernoma, is a type of venous malformation due to endothelial dysmorphogenesis from a lesion which is present at birth. A cavernoma in the brain is called a cerebral cavernous malformation or CCM. Despite its designation as a hemangioma, a cavernous hemangioma is not a tumor as it does not display endothelial hyperplasia. The abnormal tissue causes a slowing of blood flow through the cavities, or "caverns". The blood vessels do not form the necessary junctions with surrounding cells, and the structural support from the smooth muscle is hindered, causing leakage into the surrounding tissue. It is the leakage of blood, referred to as hemorrhage, that causes a variety of symptoms known to be associated with the condition.

<span class="mw-page-title-main">Cavernous liver hemangioma</span> Medical condition

A cavernous liver hemangioma or hepatic hemangioma is a benign tumor of the liver composed of large vascular spaces lined by monolayer hepatic endothelial cells. It is the most common benign liver tumour, and is usually asymptomatic and diagnosed incidentally on radiological imaging or during laparotomy for other intra-abdominal issues. Liver hemangiomas are thought to be congenital in origin with an incidence rate of 0.4 – 7.3% as reported in autopsy series.

<span class="mw-page-title-main">Hemangioma</span> Medical condition

A hemangioma or haemangioma is a usually benign vascular tumor derived from blood vessel cell types. The most common form, seen in infants, is an infantile hemangioma, known colloquially as a "strawberry mark", most commonly presenting on the skin at birth or in the first weeks of life. A hemangioma can occur anywhere on the body, but most commonly appears on the face, scalp, chest or back. They tend to grow for up to a year before gradually shrinking as the child gets older. A hemangioma may need to be treated if it interferes with vision or breathing or is likely to cause long-term disfigurement. In rare cases internal hemangiomas can cause or contribute to other medical problems. They usually disappear in 10 years. The first line treatment option is beta blockers, which are highly effective in the majority of cases. Hemangiomas that form at birth are called congenital hemangiomas, while those that form later in life are called infantile hemangiomas.

<span class="mw-page-title-main">Hereditary neurocutaneous angioma</span> Medical condition

Hereditary neurocutaneous angioma is a rare genetic disorder characterized by the appearance of angiomas on cutaneous and neurological areas of the body in multiple members of a single family.

References

  1. Angiomatosis at the U.S. National Library of Medicine Medical Subject Headings (MeSH)
  2. 1 2 3 Howat, AJ; Campbell, PE (October 1987). "Angiomatosis: a vascular malformation of infancy and childhood. Report of 17 cases". Pathology. 19 (4): 377–82. doi:10.3109/00313028709103887. PMID   3444663. S2CID   23743347.
  3. "angiomatosis" at Dorland's Medical Dictionary
  4. 1 2 "Von Hippel–Lindau Disease (VHL) Information Page". www.ninds.nih.gov. National Institute of Neurological Disorders and Stroke.