Wednesday, June 19, 2019

Congenital Nail Dystrophy

The patient is a nine month old fraternal twin with mild developmental delays. No other pertinent history

She has opaque toenails that grow at an upward angle from the nail bed since birth. The great toenails continue to have an increasingly severe upward slant so that they are almost at a 90 degree angle to the nail bed. The nail plates show no thickening. Her fingernails are all normal. The skin of the feet and the rest of the body appear normal. No peeling or scale. No rash. The foot anatomy is grossly otherwise normal. The twin does not have the same condition. The parents are unable to put shoes on her because she seems to be in pain from her toenails. She does not seem bothered by socks or soft slippers. She is not walking yet, but should be within 2-3 months. they cut the nails short, but it doesn't offer enough relief to use shoes.  

Clinical Image:

Has anyone seen a similar patient?  What are your thoughts?

Note:  The infant will be seen by a dermatologist in a few days and a KOH prep will hopefully be done.


  1. Hard to tell from the one photo provided but this could be a variant of congenital malalignment of the great toenails. Is the nail plate deviated laterally?

  2. Khalifa Sharquie from Baghdad wrote: "This is onycholysis rather than nail dystrophy which is commonly caused by psoriasis even in infant and never to be fungal"

  3. Dato Cheng Ong Leng from Malaysia wrote: "It’s pincer nails, congenital type. May I know whether the other unaffected twin is a fraternal or identical twin?
    If it’s identical twin, we don’t have to worry about a genetic syndrome.
    Pincer nails are thought to be nails bigger than the plots ( nail beds) drawn out for them, hence they become curved, detached from the nail beds, and ingrowing into the surroundings, causing redness and swelling muck like paronychia.

    I suggest to treat any infections arising from this unhappy nail-skin relationship and referral to our colleagues who specialise in nails."

  4. Mary Maloney, M.D., Worcester, Massachusetts wrote: "There is a congenital nail dystrophy that resolves in 6-12 mos. One of my kids had it and in fact resolved before 1st birthday."

  5. I would guess this resolves with time, as congenital nail dystrophy is fairly common. Ruling out infection is always a good idea. Try to keep the nails cut short for comfort. Of course would monitor for any syndromic associations over time (an unaffected identical twin does not rule out a genetic condition as de novo mutations are not uncommon - especially with AD keratin mutations as in pachyonychia congenita)... we would be happy to see them at Children’s.


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