Becker nevus syndrome

Alternate Names

Hairy epidermal nevus syndrome

Definition

Becker nevus syndrome is characterized by the presence of a Becker nevus in association with underdevelopment (hypoplasia) of the breast or other skin-related, muscular, or skeletal defects, all of which usually involve the same side of the body as the nevus (ipsilateral).

Inheritance

The condition is thought to be sporadic (occurring in individuals with no history of the condition in the family).

Signs and symptoms

Becker nevus syndrome is characterized by the presence of a Becker nevus in association with underdevelopment (hypoplasia) of the breast and/or other skin-related (cutaneous), muscular, or skeletal defects, all of which usually involve the same side of the body as the nevus (ipsilateral). Breast hypoplasia affects both males and females, but is more noticeable in females.

Other muscular and skeletal abnormalities may include:

• Absence of the pectoralis major muscle (pectoral)
• Underdevelopment of the muscles of the shoulder girdle
• Abnormal curvature of the spine (scoliosis)
• Vertebral defects
• Fused ribs
• Ipsilateral shortness of a limb
• Underdevelopment of the teeth and jaws
• A "sunken chest" (pectus excavatum) or abnormally prominent chest (pectus carinatum)
• Extra (supernumerary) nipples
• Abnormally sparse hair under the armpit.

For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. 80%-99% of people have these symptoms

• Hamartoma
• Hypermelanotic macule(Hyperpigmented spots)
• Lipoatrophy(Loss of fat tissue in localized area)
• Micromelia(Smaller or shorter than typical limbs)
• Shoulder girdle muscle atrophy(Shoulder girdle muscle wasting)
• Supernumerary nipple(Accessory nipple)

30%-79% of people have these symptoms

• Aplasia/Hypoplasia of the breasts(Absent/small breasts)
• Pectus carinatum(Pigeon chest)
• Pectus excavatum(Funnel chest)

5%-29% of people have these symptoms

• Abnormality of the scrotum
• Abnormality of tibia morphology(Abnormality of the shankbone)
• Hypoplastic labia minora(Underdeveloped inner lips)
• Kyphosis(Hunched back)
• Lower limb asymmetry(Left and right leg differ in length or width)
• Rib fusion(Fused ribs)
• Scoliosis
• Spina bifida occulta
• Supernumerary ribs(Extra ribs)
• Upper limb asymmetry(Unequal size of arms)

Treatment

Treatment varies depending upon the specific symptoms present and the extent of the condition in the affected individual.

NIH genetic and rare disease info

• NIH info on Becker nevus syndrome

Becker nevus syndrome is a rare disease.