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Multiple cutaneous and mucosal venous malformations
Reviewed by Medicinsk Expert•Last updated: 2026-10-22•Condition

Multiple cutaneous and mucosal venous malformations

Multiple cutaneous and mucosal venous malformations (VMCM) is a condition characterized by the development of painful, bluish lesions on the skin and mucous membranes due to improperly formed veins.

Overview

Multiple cutaneous and mucosal venous malformations (VMCM) involve the presence of bluish patches or lesions on the skin and mucous membranes (such as the mouth and nose). These lesions are caused by venous malformations—areas where veins and other blood vessels did not develop properly. Lesions may become painful if they extend into muscles or joints, or if calcium deposits cause inflammation.

Causes

VMCM is caused by mutations in the TEK gene (also known as TIE2). This gene encodes a receptor protein that regulates blood vessel formation (angiogenesis) and maintains vessel structure by facilitating communication between endothelial cells and smooth muscle cells.

Mutations in the TEK gene result in an overactive receptor, which disrupts the communication between these cell types. This leads to abnormal blood vessels that lack sufficient smooth muscle cells, resulting in the characteristic lesions of VMCM.

Inheritance

VMCM is inherited in an autosomal dominant pattern, meaning one copy of the altered gene is sufficient to increase the risk of developing the condition. In most cases, an affected person has one parent with the condition.

Some cases involve somatic mutations—changes acquired during a person's lifetime that are present only in certain cells. Research suggests that some VMCM lesions may require both an inherited mutation and a somatic mutation to form.

Other Names

  • Mucocutaneous venous malformations
  • VMCM
  • VMCM1

Frequently Asked Questions

While many lesions are stable, patients should seek immediate medical attention if a lesion suddenly becomes significantly swollen, hard, and extremely painful, which may indicate a localized blood clot (thrombosis) within the malformation. Other urgent signs include rapid expansion of the lesion, skin breakdown or ulceration over the area, or signs of infection such as redness, warmth, and fever. These complications can lead to tissue damage or systemic issues if not treated promptly.
Because VMCM is autosomal dominant, an affected individual has a 50 percent chance of passing the TEK gene mutation to each child. Genetic counseling is recommended for prospective parents to discuss the risks and available testing options. It is important to note that the condition exhibits variable expressivity, meaning that even if a child inherits the mutation, the number, size, and severity of the lesions can differ significantly from the parent, ranging from mild skin patches to extensive mucosal involvement.
Complete removal of the underlying vascular malformation is rarely possible with cosmetic procedures alone, as the abnormal vessels are often deep and extensive. Treatments such as laser therapy or sclerotherapy may help reduce the visible bluish discoloration and shrink smaller lesions, but they are often palliative rather than curative. In cases where lesions cause significant functional impairment or severe pain, surgical resection may be considered, though this carries risks of bleeding and recurrence. Management is typically focused on symptom control rather than complete aesthetic correction.
Management often focuses on preventing trauma and reducing pressure on the lesions. Patients are advised to wear compression garments to support the affected limbs and reduce swelling, while avoiding tight clothing that might irritate the skin. For mucosal lesions in the mouth or nose, using soft-bristled toothbrushes and avoiding acidic or spicy foods can minimize irritation. Regular, low-impact exercise is generally encouraged to maintain circulation, but high-impact activities that risk bruising or injury to the lesions should be avoided.
Source: MedlinePlus, National Library of Medicine

MedlinePlus brings together authoritative health information from the National Library of Medicine (NLM), the National Institutes of Health (NIH), and other government agencies and health-related organizations.

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