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Progressive Lymphangioma of Skin

Article
Skin Care
Diseases & Conditions
+1
Contributed byLester Fahrner, MD+1 moreDec 21, 2021

What are the other Names for this Condition? (Also known as/Synonyms)

  • Acquired Progressive Lymphangioma of Skin
  • Benign Lymphangioendothelioma of Skin
  • Cutaneous Progressive Lymphangioma

What is Progressive Lymphangioma of Skin? (Definition/Background Information)

  • A lymphangioma is a benign type of tumor that develops spontaneously, due to certain abnormalities in the lymphatic system
  • Progressive Lymphangioma of Skin is an acquired condition that manifests as a benign vascular tumor of the skin and subcutaneous tissues in adults
  • Most tumors are present on the lower limb as a slow-growing pigmented skin patch/plaque. In many cases, Progressive Lymphangiomas of Skin do not show any signs and symptoms; however, some present pain and itching sensations
  • A surgical excision of the tumor is considered curative. The prognosis for Progressive Lymphangioma of Skin is excellent with suitable treatment, although some are known to recur after treatment

Who gets Progressive Lymphangioma of Skin? (Age and Sex Distribution)

  • Progressive Lymphangioma of Skin is an extremely rare tumor of the skin seen in adults; much less than 50 cases have been identified/reported
  • An age range of 17-90 years has been noted, though the tumor typically affects middle-aged and older adults
  • In some cases, teens and young adults have been reported with this tumor
  • Both males and females are affected and no gender predilection is observed
  • No racial or ethnic preference has been reported

What are the Risk Factors for Progressive Lymphangioma of Skin? (Predisposing Factors)

The risk factors for Progressive Lymphangioma of Skin may include:

  • Insect and tick bites
  • Any surgical procedure causing trauma to the skin
  • Radiation therapy administered for any medical condition such as cancer
  • Hormonal imbalances in the body, especially following puberty

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one's chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Progressive Lymphangioma of Skin? (Etiology)

  • A Progressive Lymphangioma of Skin develops when the lymphatic system gets obstructed due to various reasons
  • The obstruction causes fluid to fill-up within the lymphatic channels, which then dilate
  • The obstruction of the lymphatic channels may be secondary to trauma, or due to iatrogenic factors (the lesion may arise from around a surgical scar)

What are the Signs and Symptoms of Progressive Lymphangioma of Skin?

The signs and symptoms of Progressive Lymphangioma of Skin may include:

  • It is a slow, but progressively growing tumor; the size range may vary from 1 to 10 cm (average size about 2 cm)
  • In the initial stages, the solitary lesion may be present as a skin patch/plaque that is red and inflamed
  • The lesion appears like a bruise on the skin; the color of the lesion may vary from red to yellow
  • It may grow to large sizes over many years; some tumors are known to be present for a few decades
  • Itching, pain, and tenderness may be noticed; hair loss may be present in some cases
  • The tumor may form anywhere on the body skin, but is commonly seen on the legs

How is Progressive Lymphangioma of Skin Diagnosed?

A diagnosis of Progressive Lymphangioma of Skin may involve:

  • Through physical exam with evaluation of complete family medical history
  • Dermoscopy: It is a diagnostic tool where a dermatologist examines the skin using a special magnified lens
  • Wood’s lamp examination: In this procedure, the healthcare provider examines the skin using ultraviolet light. It is performed to examine the change in skin pigmentation
  • Skin biopsy: A tissue biopsy is performed and sent to a laboratory for a pathological examination. The pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis
  • A differential diagnosis may be necessary prior to establishing a definitive diagnosis, by excluding tumors such as well-differentiated angiosarcoma and Kaposi's sarcoma

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Progressive Lymphangioma of Skin?

The possible complications due to Progressive Lymphangioma of Skin may include:

  • Infection from surgical interventions
  • Growth of the tumor after surgery is known to take place following an incomplete removal of the tumor
  • Adjoining structures, tissues, nerves, and organs, can be damaged during the surgery

How is Progressive Lymphangioma of Skin Treated?

Following are the treatment methods for Lymphangioma:

  • Surgical removal of the tissue mass: A surgery is the most effective method for a complete cure
  • Use of steroid and sclerosing injections into the tumor; oral prednisone therapy seems to have a beneficial effect
  • Post-operative care is important until the surgical wound heals
  • Follow-up care with regular screening and check-ups are important, to watch for any recurrences

How can Progressive Lymphangioma of Skin be Prevented?

Current medical research has not established a method of preventing Progressive Lymphangioma of Skin.

What is the Prognosis of Progressive Lymphangioma of Skin? (Outcomes/Resolutions)

  • The prognosis of Progressive Lymphangioma of Skin is generally excellent, if no additional complications or any surgical infections develop, since it is a benign condition
  • Some tumors are known to regress and reduce in size following systemic treatment with steroids; some tumors are known to shrink despite any therapy or treatment
  • Tumor recurrence following surgery is known to take place if the lymphangiomas are removed incompletely

Additional and Relevant Useful Information for Progressive Lymphangioma of Skin:

A lymphangioma is a benign type of tumor that develops spontaneously due to certain abnormalities in the lymphatic system. It is of two types - cystic lymphangioma and cavernous lymphangioma.

The following link may help you find useful additional information on lymphangioma:

https://www.dovemed.com/diseases-conditions/lymphangioma/

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On the Article

Krish Tangella MD, MBA picture
Approved by

Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team
Lester Fahrner, MD picture
Reviewed by

Lester Fahrner, MD

Chief Medical Officer, DoveMed Team

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