Summary about Disease
Young-Laor syndrome, also known as familial gingival fibromatosis with hypertrichosis, is a rare genetic disorder characterized primarily by two main features: gingival fibromatosis (overgrowth of the gums) and hypertrichosis (excessive hair growth). The gingival overgrowth can be so severe that it covers the teeth, and the hypertrichosis often affects the face, limbs, and back. Less common features may include intellectual disability, short stature, or other skeletal anomalies.
Symptoms
Gingival Fibromatosis: Excessive overgrowth of the gums, which can cover the teeth partially or entirely. This can lead to difficulty with chewing, speaking, and maintaining oral hygiene.
Hypertrichosis: Excessive hair growth on various parts of the body, including the face, limbs, and back.
Possible Intellectual Disability: Some individuals may experience varying degrees of intellectual disability.
Possible Short Stature: Some individuals may be shorter than average for their age.
Possible Skeletal Anomalies: In some cases, skeletal abnormalities may be present.
Causes
Young-Laor syndrome is a genetic disorder. It is most often inherited in an autosomal dominant manner, meaning that only one copy of the mutated gene is needed to cause the disorder. The specific genes responsible for Young-Laor syndrome have not been conclusively identified in all cases, but some research points to mutations in certain genes that play a role in hair follicle development or connective tissue growth.
Medicine Used
There is no specific medication to cure Young-Laor syndrome. Treatment focuses on managing the symptoms:
Gingivectomy: Surgical removal of the excess gum tissue. This is often repeated as the gum tissue may regrow.
Oral Hygiene: Meticulous oral hygiene is essential to prevent secondary problems like infections and tooth decay.
Hair Removal: Various methods, such as shaving, waxing, laser hair removal, or depilatory creams, can be used to manage hypertrichosis.
Other Therapies: Addressing any developmental delays or intellectual disabilities may require special education, speech therapy, occupational therapy, or other supportive services.
Is Communicable
No, Young-Laor syndrome is not communicable. It is a genetic disorder and cannot be spread from person to person.
Precautions
Since Young-Laor syndrome is a genetic condition, there are no specific precautions to prevent its occurrence in an individual already affected. Genetic counseling may be helpful for families with a history of the syndrome who are planning to have children, to understand the risk of inheritance. Furthermore, focusing on supportive care to manage the symptoms:
Consistent dental care to manage gum overgrowth and avoid infection.
Managing hair growth with safe techniques
How long does an outbreak last?
Young-Laor syndrome is not an infectious disease with outbreaks. The symptoms are chronic and persistent, with the gingival fibromatosis and hypertrichosis being present throughout the individual's life, although the severity may vary.
How is it diagnosed?
Diagnosis of Young-Laor syndrome is primarily based on clinical evaluation, including:
Physical Examination: Assessing the characteristic features of gingival fibromatosis and hypertrichosis.
Family History: Inquiring about a family history of similar symptoms.
Genetic Testing: Genetic testing may be available to look for mutations in genes associated with related conditions, although specific gene mutations causing Young-Laor syndrome are not always identified.
Dental Examination: To evaluate the extent of gum overgrowth.
Other Evaluations: Additional evaluations may be done to assess for intellectual disability, skeletal anomalies, or other associated features.
Timeline of Symptoms
Gingival Fibromatosis: Often becomes apparent in early childhood or adolescence. The rate of gum overgrowth can vary.
Hypertrichosis: May be present at birth or develop in early childhood. The extent and distribution of hair growth can change over time.
Intellectual Disability/Developmental Delays: If present, these may be apparent in infancy or early childhood.
Important Considerations
Genetic Counseling: Families with a history of Young-Laor syndrome should consider genetic counseling to understand the risk of inheritance.
Long-Term Management: Management of Young-Laor syndrome is a lifelong process that requires ongoing dental care, hair removal, and supportive services as needed.
Psychosocial Support: The visible symptoms of Young-Laor syndrome can affect self-esteem and social interactions. Providing psychosocial support and counseling is important.
Multidisciplinary Care: Care should involve a team of specialists, including dentists, dermatologists, geneticists, and possibly other specialists, depending on the specific features present.