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

• Angiokeratoma in Tuberous Sclerosis
• Fibrous Skin Tumor of Tuberous Sclerosis
• Pringle Tumor

What is Adenoma Sebaceum? (Definition/Background Information)

• Adenoma Sebaceum is a rare skin condition in which the presence of multiple skin tumors is noted in a background of the genetic disorder tuberous sclerosis
• Adenoma Sebaceum is considered a misnomer, since the tumor is neither an adenoma (a benign tumor), nor it involves the sebaceous glands (type of sweat glands). The condition is also known as Pringle Tumor
• The signs and symptoms associated with Adenoma Sebaceum include the presence of multiple tiny lesions around the nose and cheek area. No pain is generally associated with these skin lesions
• In many cases, topical applications or laser excision techniques may be used to treat the condition, mainly for cosmetic reasons. The prognosis of Adenoma Sebaceum is generally related to the severity of the underlying genetic disorder that may vary from one individual to another

Who gets Adenoma Sebaceum? (Age and Sex Distribution)

• Adenoma Sebaceum is an uncommon skin condition that may be observed in individuals of any age with tuberous sclerosis (a congenital condition)
• In most cases, the signs and symptoms appear after age 2 and are known to progress as the child grows and develops
• The condition may occur in both males and females
• Individuals of all racial and ethnic background may be affected

What are the Risk Factors for Adenoma Sebaceum? (Predisposing Factors)

The following genetic disorder is identified as a risk factor for Adenoma Sebaceum:

• Tuberous sclerosis (TS) complex: It is an inherited genetic disorder that can cause the formation of other tumor types, such as astrocytomas, rhabdomyomas, phakomas, oncocytomas, and angiofibromas, at various body locations
• Presently, no other risk factors are clearly established

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 Adenoma Sebaceum? (Etiology)

• Adenoma Sebaceum is known to occur against a backdrop of the inherited genetic disorder tuberous sclerosis
• Tuberous sclerosis: It is an autosomal dominant condition that involves the TSC1 and TSC2 gene

What are the Signs and Symptoms of Adenoma Sebaceum?

The signs and symptoms of Adenoma Sebaceum include:

• Presence of multiple small lesions on the face, typically around the nose, chin, and cheeks
• The lesions form small papules that typically appear like flesh-colored bumps on the skin
• In some cases, the papules are red or brown in color
• These skin lesions may bleed if traumatized
• The skin lesions are usually less than 1 cm in size
• There is an absence of pain and most cases are asymptomatic

The signs and symptoms of the underlying tuberous sclerosis may be observed.

How is Adenoma Sebaceum Diagnosed?

The following are the diagnostic methods to help identify Adenoma Sebaceum:

• A thorough physical examination and a complete medical history: In many cases, a diagnosis may be established through clinical exam and biopsy studies are not required
• 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 the 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 rule-out trichoepithelioma papulosum multiplex (a rare genetic disorder) may be undertaken

Note: In most cases, a skin biopsy of the lesion is not necessary. When undertaken though, it is often to rule out other tumors presenting similar symptoms.

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 Adenoma Sebaceum?

In many cases, no complications are noted because of Adenoma Sebaceum. However, in some individuals, the complications may include:

• Cosmetic issues causing emotional stress
• The skin lesions can get secondarily infected
• Trauma or plucking of the skin lesions may result in scarring
• Complications that may develop due to the underlying genetic condition
• Damage to vital nerves, blood vessels, and surrounding structures during surgery (if performed to treat the condition)

How is Adenoma Sebaceum Treated?

The treatment options for Adenoma Sebaceum may include the following procedures and measures:

• Topical medications, such as the use of mTOR inhibitors (a certain class of drugs)
• In cases where the skin lesion is present as a flat patch, a shave biopsy of skin may be performed
• Other treatment techniques include dermabrasion and laser excision
• Treatment of the underlying genetic syndrome is important

Follow-up care with regular screening and check-ups are important and recommended.

How can Adenoma Sebaceum be Prevented?

Current medical research has not established a method of preventing Adenoma Sebaceum. However, since it is associated with a genetic disorder, 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 such as tuberous sclerosis

What is the Prognosis of Adenoma Sebaceum? (Outcomes/Resolutions)

• Generally, Adenoma Sebaceum is by itself a benign condition having good prognosis with appropriate treatment
• But, since it is associated with tuberous sclerosis, the overall prognosis is based upon the severity of the signs and symptoms of the genetic disorder

Additional and Relevant Useful Information for Adenoma Sebaceum:

The following DoveMed website link is a useful resource for additional information:

https://www.dovemed.com/healthy-living/skin-disorders/