Clinical Review & Education

JAMA Pediatrics Clinical Challenge

Sudden Painless Nail Shedding

Romano Silvio Kasper, MD; Stephan Nobbe, MD; Martin Theiler, MD; Lisa Weibel, MD

A B

Figure. Findings on clinical examination. A, Fingernails showed semilunar whitish grooves in the middle of the nail plate. B, Toenails had transverse partial cracks of
the proximal nail plate with distal onycholysis.

A 7-year-old girl presented to the dermatology clinic with a 4-week history of progressive
nail changes. Her mother reported a sudden onset of brittle nails with cracks followed by WHAT IS YOUR DIAGNOSIS?
painless sloughing of nails. Initially only the fingernails had been affected, but the toenails
soon showed the same pathology. The family was con- A. Drug intake
cerned and suspected an internal disease or vitamin
Quiz at jamapediatrics.com deficiency. The child was otherwise well and no previ-
B. Kawasaki syndrome
ous skin or nail conditions were known. On clinical
examination, most fingernails showed semilunar whitish grooves in the middle of the nail
plate (Figure, A). The toenails had transverse partial cracks of the proximal nail plate with C. Vaccination reaction
distal onycholysis but normal proximal nail growth (Figure, B).
D. Hand-foot-and-mouth disease

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 Clinical Review & Education JAMA Pediatrics Clinical Challenge

Diagnosis
D. Hand-foot-and-mouth disease (HFMD)
Discussion
Onychomadesis describes the complete shedding of the nail plate
from the nail bed, occurring after a sudden growth arrest of the nail
matrix (Figure, B). As a milder variant, Beau lines may be seen pre-
senting as semilunar transverse grooves of the nail plate (Figure, A).
While nail changes can be an important marker of genetic condi-
tions or internal disease in pediatric patients, they are usually asso-
ciated with additional changes of the skin, mucous membranes, or
hair. Although often suspected by parents, onychomycosis is rare
overall in young children.1 Nail dystrophy in childhood is often due
to eczema or paronychia affecting the nail matrix. Onycholysis and
hyperkeratosis of the nail may be the result of subungual warts.2
Acute onychomadesis is often considered to be idiopathic, but vari-
ous infectious triggers have been described.
The history of our patient revealed HFMD 7 weeks before the
onset of the nail changes. It is a rather common infection in chil-
dren caused by coxsackievirus, a member of the family of entero-
viruses. Small epidemic outbreaks are usually reported in autumn
or spring. The condition is characterized by low-grade fever and an
acute vesicular eruption of the hands, feet, buttocks, oral cavity, lips,
and perioral area. The blisters are usually small, oval shaped, and gray-
ish with an erythematous border in combination with a vesicular and
erosive stomatitis. However, the clinical presentation is very vari-
able, with some children presenting with only a few blisters and oth-
ers with generalized exanthema (eczema coxsackium).3
Since the first 5 cases reported in 2000,4 HFMD has come to
be associated with nail matrix arrest and onychomadesis. Several lo-
cal epidemics of HFMD with up to 220 patients affected by onycho-
madesis have been reported.5 Several strains were identified, most
commonly coxsackievirus B1 as in Spain6 and coxsackievirus A6 as
in Japan.7 The mechanism for post-HFMD nail matrix arrest re-
mains unclear. No relationship seems to exist between the severity
of HFMD, location of blisters, and fever. One group was able to de-
tect coxsackievirus A6 DNA extracted from nail fragments in chil-
dren with onychomadesis, supporting the idea of direct nail matrix
damage due to virus replication.8 Others have discussed second-
ary appearance due to inflammation of the nail matrix.9 Nail changes
typically appear 3 to 10 weeks after the onset of infection. During
the North American HFMD epidemic in 2011-2012, secondary nail
changes were noted in approximately one-quarter of cases.3 Oth-
ers report higher incidences of up to 70% when coxsackievirus is
involved.6 To our knowledge, no reports of onychomadesis have
been reported when other enteroviruses such as enterovirus 71 were
the cause of HFMD. Other causes for acute onychomadesis include
infections involving high fever (norovirus infection, swine flu, scar-
let fever), Kawasaki syndrome, drug intake (anticonvulsants, anti-
biotics, chemotherapy agents, and retinoids), acute paronychia, Ste-
vens-Johnson syndrome, systemic lupus erythematosus, pemphigus
vulgaris, and epidermolysis bullosa.2
Onychomadesis following HFMD is a self-limiting condition with
subsequent normal nail regrowth within weeks. No specific treat-
ment is indicated. Affected patients and their parents should be re-
assured about the benign nature of this appearance.

ARTICLE INFORMATION
Author Affiliations: Department of Dermatology,
University Hospital Zurich, Zurich, Switzerland
(Kasper, Nobbe, Theiler, Weibel); Department of
Dermatology, University Childrens Hospital Zurich,
Zurich, Switzerland (Theiler, Weibel).
2. Chu DH, Rubin AI. Diagnosis and management of
nail disorders in children. Pediatr Clin North Am.
2014;61(2):293-308.
3. Mathes EF, Oza V, Frieden IJ, et al. Eczema
coxsackium and unusual cutaneous findings in an
enterovirus outbreak. Pediatrics. 2013;132(1):
7. Miyamoto A, Hirata R, Ishimoto K, et al.
An outbreak of hand-foot-and-mouth disease
mimicking chicken pox, with a frequent association
of onychomadesis in Japan in 2009: a new
phenotype caused by coxsackievirus A6. Eur J
Dermatol. 2014;24(1):103-104.

Corresponding Author: Lisa Weibel, MD, e149-e157. 8. Osterback R, Vuorinen T, Linna M, Susi P,
Department of Dermatology, University Childrens 4. Clementz GC, Mancini AJ. Nail matrix arrest Hyypi T, Waris M. Coxsackievirus A6 and hand,
Hospital Zurich, Steinwiesstrasse 75, 8032 Zrich, following hand-foot-mouth disease: a report of five foot, and mouth disease, Finland. Emerg Infect Dis.
Switzerland (lisa.weibel@kispi.uzh.ch). children. Pediatr Dermatol. 2000;17(1):7-11. 2009;15(9):1485-1488.

Section Editor: Samir S. Shah, MD, MSCE.
Conflict of Interest Disclosures: None reported.
REFERENCES
5. Davia JL, Bel PH, Ninet VZ, et al. Onychomadesis
outbreak in Valencia, Spain associated with hand,
foot, and mouth disease caused by enteroviruses.
Pediatr Dermatol. 2011;28(1):1-5.
9. Haneke E. Onychomadesis and hand, foot and
mouth disease: is there a connection? Euro Surveill.
2010;15(37).

1. Gupta AK, Sibbald RG, Lynde CW, et al.
Onychomycosis in children: prevalence and
treatment strategies. J Am Acad Dermatol. 1997;36
(3, pt 1):395-402.
6. Cabrerizo M, De Miguel T, Armada A,
Martnez-Risco R, Pousa A, Trallero G.
Onychomadesis after a hand, foot, and mouth
disease outbreak in Spain, 2009. Epidemiol Infect.
2010;138(12):1775-1778.

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