Familial Multiple Fibrofolliculoma

Familial Multiple Fibrofolliculoma

Article
Skin Care
Diseases & Conditions
+3
Contributed byLester Fahrner, MD+1 moreSep 05, 2021

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

  • Congenital Multiple Fibrofolliculoma
  • Inherited Multiple Fibrofolliculoma

What is Familial Multiple Fibrofolliculoma? (Definition/Background Information)

  • Familial Multiple Fibrofolliculoma is a highly uncommon genetic disorder affecting the skin. In this condition, numerous asymptomatic lesions (or fibrofolliculomas) may be observed on the body. The symptoms are usually noted in adulthood
  • Familial Multiple Fibrofolliculoma is described as a genodermatosis, meaning a hereditary skin condition that may run in the families. Presently, there are no well-established treatment measures available for this benign condition. The prognosis of individuals with this condition may vary from one to another

Who gets Familial Multiple Fibrofolliculoma? (Age and Sex Distribution)

  • Familial Multiple Fibrofolliculoma is an extremely rare congenital disorder, with an estimated prevalence of less than 1:1,000,000
  • The presentation of symptoms typically occurs in adulthood
  • Both males and females may be affected
  • Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Familial Multiple Fibrofolliculoma? (Predisposing Factors)

  • A positive family history may be an important risk factor, since Familial Multiple Fibrofolliculoma can be inherited
  • Currently, no other risk factors have been clearly identified for the disorder

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 Familial Multiple Fibrofolliculoma? (Etiology)

  • The exact genetic mutation causing Familial Multiple Fibrofolliculoma is presently unknown
  • The condition is reportedly inherited in an autosomal dominant manner

Autosomal dominant pattern of inheritance: Autosomal dominant conditions are traits or disorders that are present when only one copy of the mutation is inherited on a non-sex chromosome. In these types of conditions, the individual has one normal copy and one mutant copy of the gene. The abnormal gene dominates, masking the effects of the correctly function gene. If an individual has an autosomal dominant condition, the chance of passing the abnormal gene on to their offspring is 50%. Children, who do not inherit the abnormal gene, will not develop the condition, or pass it on to their offspring.

What are the Signs and Symptoms of Familial Multiple Fibrofolliculoma?

The signs and symptoms of Familial Multiple Fibrofolliculoma may vary among affected individuals, and include the following:

  • Presence of multiple lesions (or fibrofolliculomas) on skin; these may occur anywhere in the body
  • A fibrofolliculoma is a rare and slow-growing benign tumor of the hair follicles
  • The skin lesions are typically painless and non-itchy
  • The lesions may be in the form of papules (elevated skin areas that are well-circumscribed) and plaques (slightly elevated thickened skin areas of varying shapes)
  • Several lesions may be present on the face in a random manner
  • Hair loss may be noted

How is Familial Multiple Fibrofolliculoma Diagnosed?

Familial Multiple Fibrofolliculoma is diagnosed on the basis of the following information:

  • Complete physical examination and thorough medical history evaluation
  • Assessment of the presenting signs and symptoms
  • 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 skin 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 to eliminate other conditions that present similar signs and 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 Familial Multiple Fibrofolliculoma?

The complications of Familial Multiple Fibrofolliculoma may include:

  • Cosmetic concerns due to multiple lesions, particularly on the face
  • Secondary infection of the lesions
  • Scarring, if the lesions rupture and ulcerate

Complications may occur with or without treatment, and in some cases, due to treatment also.

How is Familial Multiple Fibrofolliculoma Treated?

There are no well-defined treatments or cure for Familial Multiple Fibrofolliculoma since it is a genetic condition.

  • The treatment is usually given to manage the signs and symptoms and any complication that develops, which may involve topical medications and systemic therapy
  • Surgical destruction with cautery or lasers might be attempted
  • Regular medical screening at periodic intervals with tests and physical examinations are recommended

How can Familial Multiple Fibrofolliculoma be Prevented?

Currently, Familial Multiple Fibrofolliculoma may not be preventable, since it is a genetic disorder.

  • 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 Familial Multiple Fibrofolliculoma? (Outcomes/Resolutions)

  • The prognosis of Familial Multiple Fibrofolliculoma is dependent upon the severity of the signs and symptoms and associated complications, if any
  • Individuals with mild conditions have better prognosis than those with severe symptoms and complications. Typically, the prognosis may be assessed on a case-by-case basis

Additional and Relevant Useful Information for Familial Multiple Fibrofolliculoma:

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

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

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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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