What is Clouston Syndrome?

Clouston Syndrome is a form of ectodermal dysplasia that is characterized by abnormalities of the skin, nails and hair.  Early indications generally start in infancy and may include aberrant nail growth and sparse, wiry, brittle, spotty, and pale scalp hair.

People who have this condition may also have palmoplantar hyperkeratosis (thickened skin on the palms of the hands and soles of the feet), hyperpigmentation of skin (especially over joints) and/or clubbing of the fingers.  Clouston syndrome is the result of changes (mutations) in the GJB6 gene and is hereditary in an autosomal dominant manner.  Clouston syndrome is a genetic condition, which then means that it is caused by one or more genes not working properly.  It occurs in approximately 1 per 500,000 live births.  The disorder is mostly seen in individuals with French Canadian ancestry, but all ethnic and racial groups can be affected.

Symptoms of Clouston Syndrome

Nails

• Thick, discolored
• Underdeveloped
• Severe malformation
• Spontaneous separation of the nail plate starting at the distal free margin and progressing proximally.

Skin

• Normal sweating
• Hyperpigmentation on the knuckles, elbows, axillae, areolae, pubic area
• Abnormal thickening of the skin of the palms and soles (palmoplantar hyperkeratosis) that gets worse with age

Hair

• Fine, brittle, slow-growing
• Total balding (alopecia)
• Fine, brittle, slow-growing
• Sparse eyebrow
• Sparse eyelashes
• Reduced hair strength
• Missing in spots for males
• Absent underarm hair

A doctor can diagnose Clouston syndrome on the basis of physical features.  Moreover, treatment is based on the signs and symptoms existing in each person.  Individuals with this condition are susceptible to immune system disturbances as well as paronychia.

Are you suffering from this condition? At The Chelsea Clinic, we can help. One of our podiatrist can assist and then recommend what treatments are best to get you back on track. Podiatrist South Kensington

Schedule an appointment here or you may call us at +44 (0) 207 101 4000. 

We hope you have a feetastic day! 

-The Chelsea Clinic and Team