Hypertrichosis lanuginosa congenita

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ORPHA:2222OMIM:145700Q84.2
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Overview

Hypertrichosis lanuginosa congenita (HLC), sometimes referred to as 'congenital generalized hypertrichosis' or historically and colloquially as 'werewolf syndrome,' is an extremely rare genetic condition characterized by excessive growth of fine, soft lanugo hair (the type of hair normally seen on fetuses) covering most of the body. Lanugo hair, which is typically shed before or shortly after birth in unaffected individuals, persists and continues to grow throughout life in people with HLC. The excessive hair growth can cover nearly the entire body surface, including the face, ears, and trunk, though the palms of the hands and soles of the feet are usually spared. Hair length can reach several centimeters and may be lighter or darker depending on the individual's background. The condition primarily affects the integumentary system (skin and hair). Aside from the generalized hypertrichosis, individuals with HLC typically do not have other significant physical abnormalities or intellectual impairment, distinguishing it from other hypertrichosis syndromes that may be associated with gingival hyperplasia or other systemic features. However, some cases have been reported with associated dental anomalies. The excessive hair growth can cause significant psychosocial distress and impact quality of life. There is currently no cure for hypertrichosis lanuginosa congenita. Management is primarily symptomatic and focuses on hair removal techniques, including shaving, waxing, depilatory creams, electrolysis, and laser hair removal, though results vary and regrowth is common. Psychological support and counseling are important components of care, particularly for children and adolescents. Genetic counseling is recommended for affected families. Research into the genetic basis of HLC is ongoing, with some families showing linkage to specific chromosomal regions, but the precise causative gene has not been definitively established in all cases.

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Inheritance

Autosomal dominant

Passed on from just one parent; each child has about a 50% chance of inheriting it

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Hypertrichosis lanuginosa congenita.

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Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

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Common questions about Hypertrichosis lanuginosa congenita

What is Hypertrichosis lanuginosa congenita?

Hypertrichosis lanuginosa congenita (HLC), sometimes referred to as 'congenital generalized hypertrichosis' or historically and colloquially as 'werewolf syndrome,' is an extremely rare genetic condition characterized by excessive growth of fine, soft lanugo hair (the type of hair normally seen on fetuses) covering most of the body. Lanugo hair, which is typically shed before or shortly after birth in unaffected individuals, persists and continues to grow throughout life in people with HLC. The excessive hair growth can cover nearly the entire body surface, including the face, ears, and trunk,

How is Hypertrichosis lanuginosa congenita inherited?

Hypertrichosis lanuginosa congenita follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Hypertrichosis lanuginosa congenita typically begin?

Typical onset of Hypertrichosis lanuginosa congenita is neonatal. Age of onset can vary across affected individuals.