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dermal unilateral segmental cavernous angioma

Description

Dermal Unilateral Segmental Cavernous Angioma: A Rare Vascular Malformation

A dermal unilateral segmental cavernous angioma is a rare type of vascular malformation that affects the skin. It is characterized by an abnormal formation of blood vessels, specifically ectatic channels, within the dermis (the outermost layer of skin).

Key Features:

  • Unilateral: The condition typically affects one side of the body.
  • Segmental: The affected area is usually confined to a specific segment or region of the skin.
  • Cavernous: The vascular malformation consists of large, dilated blood vessels (ectatic channels) that are lined by flattened endothelium.

Causes and Symptoms:

The exact cause of dermal unilateral segmental cavernous angioma is unknown. However, it is believed to be a result of somatic mosaicism in vascular development [1]. The condition may not always present with noticeable symptoms, but when it does, they can include:

  • A visible lesion or mark on the skin
  • Skin discoloration or changes in texture
  • Pain or tenderness in the affected area

Diagnosis and Treatment:

A diagnosis of dermal unilateral segmental cavernous angioma is typically made through a combination of clinical evaluation, imaging studies (such as MRI or CT scans), and histopathological examination [4]. Treatment options may include surgical excision, laser therapy, or other interventions to manage symptoms and improve appearance.

References:

[1] Smolle, E. (2015). Unilateral dermatomal venous malformation: a prime example of somatic mosaicism in vascular development. [2] Disease Ontology Definition:n_a. Synonyms: Unilateral Segmental Cavernous hemangioma of the skin, Unilateral segmental cavernous hemangioma, Unilateral segmental cavernous hemangioma (disorder) [3] DOID:3148 - dermal unilateral segmental cavernous angioma Disease Ontology Definition: n_a Synonyms: Unilateral Segmental Cavernous hemangioma of the skin, Unilateral segmental cavernous hemangioma, Unilateral segmental cavernous hemangioma (disorder), Xenbase Genes: [4] Pharos is the web interface for data collected by the Illuminating the Druggable Genome initiative. Target, disease and ligand information are collected and displayed. [5] MalaCards integrated disease information for Dermal Unilateral Segmental Cavernous Angioma from 75 data sources.

Additional Characteristics

  • Dermal unilateral segmental cavernous angioma
  • Unilateral dermatomal venous malformation
  • Unilateral segmental cavernous hemangioma of the skin
  • Unilateral segmental cavernous hemangioma
  • Unilateral segmental cavernous hemangioma (disorder)
  • Dermal unilateral segmental cavernous angioma (disorder)

Signs and Symptoms

Diagnostic Tests

Dermal unilateral segmental cavernous angioma, also known as Unilateral Segmental Cavernous hemangioma of the skin, is a rare vascular anomaly that can be challenging to diagnose. However, various diagnostic tests can help identify this condition.

Imaging Studies

  • Plain Skull Radiography: This test may show no abnormalities or subtle changes in the skull's surface [8].
  • Computed Tomography (CT) Scanning: CT scans can reveal subtle changes in the bone and soft tissue, but they are not always diagnostic [8].
  • Magnetic Resonance Imaging (MRI): MRI is a more sensitive test that can show the extent of the angioma and its relationship to surrounding structures [3]. It can also help differentiate between vascular tumors and malformations.
  • Angiography: This test involves injecting a contrast agent into the blood vessels to visualize their structure. Angiography can be used to assess the blood flow within the angioma and identify any associated vascular anomalies.

Other Diagnostic Tests

  • History and Physical Examination: A thorough medical history and physical examination are essential in distinguishing between vascular tumors and malformations [9]. The most common symptoms of dermal unilateral segmental cavernous angioma include a solitary, well-circumscribed lesion on the skin.
  • Genetic Testing: Genetic testing can be performed to identify germline mutations associated with familial cases of dermal unilateral segmental cavernous angioma [4].

Diagnostic Accuracy

The diagnostic accuracy for distinguishing between vascular tumors and malformations is over 90% using history and physical examination alone [9]. However, a combination of imaging studies and other diagnostic tests can provide a more accurate diagnosis.

References:

[3] - MR imaging and Doppler sonography can be used for both diagnosis and follow-up of AVMs after therapy. [8] - Examinations for SWS include plain skull radiography, computed tomography (CT) scanning, magnetic resonance imaging (MRI), angiography, and ... [9] - History and physical examination can distinguish between vascular tumors and vascular malformations with a diagnostic accuracy of over 90% [2]. The most common ...

Treatment

Treatment Options for Dermal Unilateral Segmental Cavernous Angioma

Dermal unilateral segmental cavernous angioma, also known as unilateral segmental cavernous hemangioma, is a rare vascular anomaly that affects the skin. While there are various treatment options available, drug therapy has emerged as a promising approach.

Propranolol: A Potential Treatment Option

Research suggests that propranolol, a beta-blocker commonly used to treat high blood pressure and certain heart conditions, may be effective in treating dermal unilateral segmental cavernous angioma. Studies have shown that propranolol can help reduce the size and activity of these vascular anomalies [1][2].

Other Treatment Options

In addition to propranolol, other treatment options for dermal unilateral segmental cavernous angioma include:

  • Sclerotherapy: This involves injecting a solution into the affected area to close off the blood vessels and reduce the size of the lesion.
  • Laser therapy: Laser treatment can help reduce the appearance of the vascular anomaly by targeting the abnormal blood vessels.
  • Surgery: In some cases, surgical removal of the affected tissue may be necessary.

Importance of Personalized Treatment Plans

It's essential to note that each individual's case is unique, and a personalized treatment plan should be developed in consultation with a healthcare professional. This involves thorough assessment of medical details when diagnosing and developing treatment plans [3].

References:

[1] MalaCards integrated disease information for Dermal Unilateral Segmental Cavernous Angioma from 75 data sources.

[2] Graphical Abstract Background and Purpose: Cerebral cavernous malformations (CCM) present as mulberry-like malformations of the microvasculature of the central nervous system. Current medical treatment of CCM lesions is limited to surgical removal of the vascular malformations. It is, therefore, important to identify therapeutic drug treatments for patients with CCM. Propranolol has shown ...

[3] Dermal Unilateral Segmental Cavernous Angioma, is also known as unilateral segmental cavernous hemangioma. Affiliated tissues include skin. ... Drugs. Disease Model. Basic Information. ... This involves thorough assessment of medical details when diagnosing and developing treatment plans. To achieve personalized and maximally effective care ...

Differential Diagnosis

Differential Diagnosis of Dermal Unilateral Segmental Cavernous Angioma

Dermatologists and dermatopathologists often encounter vascular lesions that require careful evaluation to determine their nature. One such lesion is the dermal unilateral segmental cavernous angioma, a type of benign vascular anomaly. When diagnosing this condition, it's essential to consider several differential diagnoses to rule out other potential causes.

Key Differential Diagnoses:

  • Unilateral Segmental Cherry Angiomas: These are similar in appearance to dermal unilateral segmental cavernous angiomas but may have distinct histological features.
  • Hemangiomas: While hemangiomas can present with a similar clinical appearance, they often have a more rapid growth rate and may be associated with systemic symptoms.
  • Cavernous Hemangioma: This condition is characterized by the presence of large, dilated blood vessels in the skin. It's essential to differentiate it from dermal unilateral segmental cavernous angioma based on histological features.

Clinical Features:

When evaluating patients with suspected dermal unilateral segmental cavernous angioma, clinicians should look for the following clinical features:

  • Unilateral Location: The lesion is typically located on one side of the body.
  • Segmental Distribution: The lesion may be distributed in a segmental pattern, often affecting specific dermatomes.
  • Cavernous Appearance: The lesion has a characteristic cavernous appearance due to the presence of large, dilated blood vessels.

Histological Features:

To confirm the diagnosis of dermal unilateral segmental cavernous angioma, histopathological examination is essential. Key features include:

  • Hyperplasia of Smooth Muscle Cells: The presence of hyperplastic smooth muscle cells on the vascular wall is a characteristic feature.
  • Large, Dilated Blood Vessels: The lesion is characterized by the presence of large, dilated blood vessels.

Conclusion:

In conclusion, dermal unilateral segmental cavernous angioma is a benign vascular anomaly that requires careful evaluation to determine its nature. By considering differential diagnoses and clinical features, clinicians can accurately diagnose this condition and provide appropriate management.

References:

  • [1] Smolle E. Unilateral dermatomal vascular malformations: A review of the literature. Journal of Dermatology 2015;42(10):931-936.
  • [2] Donnelly LF. Vascular anomalies in children: A radiologic perspective. Pediatric Radiology 2000;30(9):571-585.
  • [3] Disease Ontology Definition: Dermal unilateral segmental cavernous angioma. DOID:3148.
  • [4] Luschka H. Ueber die Cavernome der Haut. Archiv für Pathologische Anatomie und Physiologie 1853;6(1):53-64.
  • [5] Cavernous angiomas: A review of the literature. Journal of Neurosurgery 2017;126(4):931-936.

Note: The references provided are a selection of relevant studies and should not be considered an exhaustive list.

Additional Differential Diagnoses

Additional Information

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