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

• Cutaneous Fibrous Papule
• Dermal Angiofibroma
• Syndromal Angiofibroma of Skin

What is Angiofibroma of Skin? (Definition/Background Information)

• Angiofibroma of Skin is a common benign tumor of the skin that is usually present on the nose or cheeks. These tumors are usually seen during middle-age
• There are no clear risk factors identified for solitary tumors, but multiple Angiofibromas of Skin are seen in the context of certain genetic disorders such as tuberous sclerosis (TS) and neurofibromatosis type 2 (NF2)
• The signs and symptoms associated with Angiofibroma of Skin include the presence of small-sized and slow-growing bumps on the face. No pain is generally associated with these skin lesions
• In a majority of cases, surgical procedures or laser excision techniques may be used to treat the condition. The prognosis for Angiofibroma of Skin is excellent with suitable treatment, since these are benign tumors
• However, when these tumors occur in the context of certain underlying genetic disorder, then the overall prognosis depends upon the severity of the genetic disorder

There are various types of Angiofibroma of Skin that may be determined based on tumor sample examination by a pathologist under the microscope. The different types include:

• Hypercellular Angiofibroma of Skin: In this type, the tumor shows increased cellularity (consisting of increased number of fibroblasts)
• Clear Cell Angiofibroma of Skin: Here, the tumor has clear-to-foamy cell type
• Pigmented Angiofibroma of Skin: This tumor has increased number of melanophages, which may be mistaken for a nevus/mole
• Pleomorphic Angiofibroma of Skin: The tumor cells can have marked variation in nuclear size. This may resemble a malignant sarcoma resulting in a misdiagnosis
• Granular Angiofibroma of Skin: The tumor cells consist of granular cytoplasm, which may be confused with a granular cell tumor

Who gets Angiofibroma of Skin? (Age and Sex Distribution)

• Angiofibroma of Skin is a relatively common tumor that is observed typically in middle-aged adults. However, a wide age range of individuals may be affected
• The tumor is known to occur in both males and females
• Individuals of all racial and ethnic background may be affected; no geographical preference is also observed

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

In many cases of solitary tumors, the risk factors for Angiofibroma of Skin are generally unknown. When multiple tumors are present, the following genetic disorders have been identified as risk factors:

• Multiple endocrine neoplasia (MEN1)
• Tuberous sclerosis (TS)
• Neurofibromatosis type 2 (NF2)
• Birt-Hogg-Dubé (BHD) syndrome

When Cutaneous Angiofibromas are observed as part of a syndrome, these tumors are called Syndromal Angiofibromas of Skin.

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 Angiofibroma of Skin? (Etiology)

The exact cause of Angiofibroma of Skin is presently not well-understood.

• When numerous tumors are observed, they may be found in association with certain genetic conditions or syndromes
• When these skin lesions occur in isolation, they are almost always sporadic and no associated conditions are observed

The skin condition is not infectious and does not spread from one individual to another through contact.

What are the Signs and Symptoms of Angiofibroma of Skin?

The signs and symptoms of Angiofibroma of Skin include:

• Presence of benign tumors of size less than 1 mm; the tumor size may range anywhere from 1 mm to nearly 3 mm
• Cutaneous angiofibromas are in the form of small papules that typically appear like flesh-colored bumps on the skin
• Most of these skin lesions are solitary and observed on the nose and cheek
• There is no pain from the tumor and a majority are asymptomatic
• There may be multiple skin tumors when it is present as part of a genetic disorder. In such cases, the symptoms of the underlying genetic disorder may be observed

How is Angiofibroma of Skin Diagnosed?

The following are the diagnostic methods to help identify Angiofibroma of Skin:

• A thorough physical examination and a complete medical history (including 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
• Genetic mutational analysis to determine the presence of any associated genetic syndrome
• Skin biopsy: A skin 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 to eliminate the following tumors is often considered, before arriving at a definitive diagnosis:

• Pleomorphic fibroma
• Pearly penile papules (on penis)
• Sclerosing nevus
• Capillary hemangioma
• Fibrofolliculoma
• Adenoma sebaceum of tuberous sclerosis (multiple tumors in a background of TS)

Note:

• There are various histological subtypes of Angiofibroma of Skin that may be established by a pathologist on examination of tumor specimen. These subtypes can make the diagnosis challenging
• On clinical examination, Angiofibroma of Skin can at times resemble a very early, small basal cell carcinoma (a malignant skin condition)

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 Angiofibroma of Skin?

Significant complications are not known to typically arise from Angiofibroma of Skin. In some cases, the complications may include:

• Cosmetic issues causing emotional stress
• In rare cases, local recurrence of the angiofibroma following its removal
• Complications that may develop from the presence of underlying genetic condition (if any)
• Damage to vital nerves, blood vessels, and surrounding structures during surgery

How is Angiofibroma of Skin Treated?

The treatment options for Angiofibroma of Skin may include the following procedures and measures:

• Topical medications such as the use of mTOR inhibitors (a certain class of drugs). This treatment would only be used in a setting of multiple lesions being associated with systemic disease
• In cases where the skin lesion is present as a small solitary papule, a shave biopsy of skin may be performed
• Other techniques might include simple electrodessication or laser ablation
• Treatment of the underlying genetic syndrome, if any
• Post-operative care is important until the surgical wound heals
• Follow-up care with regular screening and check-ups are important

How can Angiofibroma of Skin be Prevented?

Current medical research has not established a method of preventing Angiofibroma of Skin. However, in case it is associated with certain genetic disorders, then the following may be considered:

• Genetic testing of the expecting parents (and related family members) and prenatal diagnosis (molecular testing of the fetus during pregnancy) may help in understanding the risks better during pregnancy
• If there is a family history of the condition, then genetic counseling will help assess risks before planning for a child
• Active research is currently being performed to explore the possibilities for treatment and prevention of inherited and acquired genetic disorders

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

• Angiofibroma of Skin is a common and benign tumor. If treated appropriately, the prognosis is usually excellent and recurrences are not observed
• If these tumors are associated with certain genetic disorders, then the overall prognosis is based upon the severity of the signs and symptoms of the specific genetic disorder

Additional and Relevant Useful Information for Angiofibroma of Skin:

Please visit our Cancer & Benign Tumors Health Center for more physician-approved health information:

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