Periodic Shedding of the Nails

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

1/14/14

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:

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.