Abstract: 2 year old
girl with seven month history of nail shedding
HPI: This otherwise
healthy 2 year-old girl has been losing nails since about 18 months of
age. By history, the nails turn black
and then are shed. The patient's father
says his small toe nail sheds periodicaly and his father may have a nail
dystrophy, also. This grandfather has a
muscular disorder.
OE: There are six or
seven nails with which shoe subungual hemorrhage, onycholysis, nail dystrophy
or absent nails. Fingers are more
affected than toenails, however, many of the toenails are dystrophic.
Clinical Photos:
12/10/13
Here is a two week f/u of nail direcctly about this photo. It's amazing how quickly the nails of young children grow.
Diagnosis:
Diagnosis:
The clinical picture is consistent with what has been called
Periodic Shedding of the Nails (PPS).
There are only two articles on this entity and both are difficult to
obtain. It is possible that what was
called PPS is really a localized variant of Epidermolysis Simplex. Since this is such a young child, the true nature of the disorder may become evident with the passage of time.
Discussion: We will try to obtain some opinions from experts
who may have some experience here. It is
likely that this is autosomal dominant with varying degrees of penetrence. It may improve with age. Trauma probably plays a role in damaging the
nail bed, so one wonders if there is some defect of the nail matrix or bed. The finger pad erosion pictured above may indicate that the problem is more wide-spread than the nail matrix.
References
1. Cutis. 1980 Jun;25(6):622-3.
Familial dystrophic periodic shedding of the nails.
Martin S, Rudolph AH.
Abstract A patient
with an autosomal dominant nail dystrophy characterized by periodic,
asymptomatic shedding of the nails followed by regrowth is described herein.
This highly penetrant disorder is similar to two earlier cases found in the
dermatologic literature.
2. Br J Dermatol.
1973 May;88(5):497-8.
Periodic shedding of the nails.
Main RA.
3. Localized epidermolysis bullosa simplex (Weber-Cockayne
type).
Villaseñor-Park J, English JC. J Pediatr Adolesc Gynecol.
2011 Dec;24(6):410-2.
I have seen a few cases of recurrent nail dystrophy with periungual erosions resulting in shedding of the affected nails in toddlers and adolescents. I considered candidiasis, onychomycosis, and traumatic nail dystrophy in the ddx. Unfortunately, most of the cases were lost to follow up.
ReplyDeletefrom Dr. Eckart Haneke:
ReplyDeletethis is certainly a rare and somehow perplexing case.
The hemorrhages in the finger nails are not in line with a simplex type of epidermolysis bullosa as the subungual tissue is protected by the overlying nail. The transverse leukponychia running parallel to the free margin of the proximal nail fold is against an internal cause. My question is whether or not the child bites on the nail, not in the sense of the common onychophagia, but more like onychodaknomania. this also happens in children. The toenails are not invovled in children above the age when they are still able to reach their toes with their mouth.
Otherwise, the nail shedding syndrome is an option.
from Jonathon Karnes, Augusta, Maine: I’d think of recent hand foot and mouth infection with some epidemic serotypes causing onychomadesis. This has only been recently observed. I’ve seen half a dozen cases in the last year. Seems more likely than EB. Here are a couple articles I could quickly find.
ReplyDeleteComment from a pediatric dermatologist: "I haven’t seen chronic onychomadesis unless there was chronic trauma. I have seen nail only patients with acral DDEB. They do have onychomadesis at times and then thickened nails. The rather abrupt onset seems odd for DDEB. I guess it is possible that minimal trauma is a problem because of underlying DDEB. I would make sure no trauma and then wait it out a bit and see how it evolves."
ReplyDeletecomment from a dermatologist with a special interest in nails: "
ReplyDeletei've never seen this.
i see plenty of idiopathic onychomadesis, but they do not have the other clinical features of this case, and they are usually limited to one or a few nails in older individuals. this seems different.
the erosion and hemorrhage are suspicious for blistering, but i would defer to KW on this point. i haven't seen kiddos present to me for this unless they already had dx of EB and, even then, it's only been a few patients."