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

• LC (Lymphangioma Circumscriptum)
• Microcystic Lymphatic Malformation

What is Lymphangioma Circumscriptum? (Definition/Background Information)

• Lymphangioma Circumscriptum (LC) can be described as small clusters of blisters filled with lymph fluid. Lymphangioma Circumscriptum is the most common type of lymphangioma affecting the skin. A lymphangioma is a benign malformation that develops spontaneously, due to certain abnormalities in the lymphatic system
• In general, when lymphangiomas occur deep in the skin tissues, they are called cystic or cavernous lymphangiomas. A lymphangioma that occurs on the surface of the skin (or superficially) is called Lymphangioma Circumscriptum
• The tumor can occur either before birth (congenital), or shortly after birth (due to physiological factors). The cause of Lymphangioma Circumscriptum is due to the obstruction of lymphatic vessels and consequent accumulation of lymph fluid
• The skin presentations include the appearance of painless, swollen, fluid-filled masses that are benign. Bleeding and ulceration may be recurrently observed
• The treatment for Lymphangioma Circumscriptum may include surgery, which may be followed by additional therapies. In some cases, skin restoration (cosmetic) procedures may have to be undertaken
• However, the prognosis of Lymphangioma Circumscriptum is generally good on a surgical excision and removal of the complete lesion. However, the prognosis may also be dependent upon other factors, such as recurrence of the lesions and/or presence of other underlying disorders, etc.

Who gets Lymphangioma Circumscriptum? (Age and Sex Distribution)

• Lymphangioma Circumscriptum usually forms before the birth of the child (during the fetal stage), or within a few years of birth
• However, the condition may be acquired during adulthood too
• Both males and females may be affected by this condition
• No specific preference for any race or ethnicity is noted

What are the Risk Factors for Lymphangioma Circumscriptum? (Predisposing Factors)

The specific risk factors of Lymphangioma Circumscriptum are unidentified. Some tumors have been observed along-with the following conditions:

• Mafucci syndrome
• Cobb syndrome
• Becker’s nevus

In some individuals, it is reported to be acquired due to the following (iatrogenic) factors:

• Surgery to the region
• Radiation treatment to the area

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 Lymphangioma Circumscriptum? (Etiology)

• Lymphangioma Circumscriptum may develop when the lymphatic system gets obstructed due to various reasons as the fetus grows and develops
• The obstruction causes fluid to fill-up within the lymphatic channels, which then dilate. The lesion continues to grow after the baby is born (growing larger)
• The reason for the obstruction of the lymphatic channels may be due to genetic factors, and/or physiological/embryological abnormalities
• In some individuals, the collection and of lymphatic fluid on the skin, with an absence of lymph drainage (secondary to certain internal or external factors), may be the reason for the formation of Lymphangioma Circumscriptum

What are the Signs and Symptoms of Lymphangioma Circumscriptum?

The signs and symptoms of Lymphangioma Circumscriptum may include:

• A very prominent, irregularly-shaped and scattered pattern of red-to-pink colored, bulging fluid-filled masses on the skin. This is the most typical sign of the lesion
• The swollen lesions may have a smooth surface, and appear like a collection of bubbles
• If they are damaged, it can ooze clear to milky fluid
• Bleeding and ulceration may be noticed; in rare cases, an involvement of the deep skin tissues may be observed
• With time, the skin over the tumor may form to be wart-like and the color may turn to deep brown
• In some cases, the simultaneous presence of skin lesions and deep-seated lymphangiomas (particularly in the chest, mediastinum, or urinary bladder region) may be observed
• The most common location of the tumor is the shoulder, neck, and the skin around the armpits
• Some other sites for Lymphangioma Circumscriptum include the upper arms and thighs, eyes (eyelids and conjunctiva), mouth (tongue), and the genitalia
• There may be an absence of pain at the location, since most tumors are painless

How is Lymphangioma Circumscriptum Diagnosed?

A diagnosis of Lymphangioma Circumscriptum may involve the following tests and exams:

• 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
• Ultrasonography and angiographic studies of affected region
• CT scan of the affected region
• Genetic tests and analysis
• Tissue biopsy of the affected region: 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
• Amniocentesis or chorionic villus sampling may be performed during pregnancy, if systemic involvement is extensively noted to determine possible associated syndrome

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 Lymphangioma Circumscriptum?

The possible complications of Lymphangioma Circumscriptum (LC) include:

• The development of secondary bacterial and fungal infections at the site
• Infection during surgical intervention, since multiple surgeries may be necessitated
• Growth of the lesion after surgery, due to an incomplete removal; recurrence of LC is commonly observed
• Adjoining structures, tissues, nerves, and organs, can be damaged during surgery
• Complications that may develop due to underlying conditions/disorders

How is Lymphangioma Circumscriptum Treated?

Following are the treatment methods for Lymphangioma Circumscriptum:

• Surgical removal of the tissue mass with reconstructive surgery (as needed): The surgery may be performed in stages and is the most effective method for a complete cure
• Combination treatment using chemotherapy and radiation therapy, particularly, if the tumor is recurrent or surgical treatment is not possible
• Use of steroids and sclerosing injections (the injections are made into the tumor)
• 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 Lymphangioma Circumscriptum be Prevented?

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

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

• The prognosis of Lymphangioma Circumscriptum is generally good, if no additional complications or any surgical infections develop
• Infrequently, some tumors are known to spontaneously regress
• Tumor recurrence following surgery is high, and therefore, recurrent episodes of bleeding and ulceration may occur
• The prognosis also depends upon the presence of any underlying syndrome and the severity of the symptoms associated with it

Additional and Relevant Useful Information for Lymphangioma Circumscriptum:

• 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 and cavernous

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

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