DOI: 10.1111/jdv.

13610 JEADV

REVIEW ARTICLE

Koilonychia: an update on pathophysiology, differential

diagnosis and clinical relevance
lez,3 N. Jellinek1,4,5,*
J. Walker,1 R. Baran,2 N. Ve
1
Department of Dermatology, The Warren Alpert Medical School of Brown University, Providence, RI, USA
2
Nail Disease Centre, Dermatology, Cannes, France
3
Division of Dermatology, Allegheny Health Network, Pittsburgh, PA, USA
4
Dermatology Professionals, Inc., East Greenwich, RI, USA
5
Division of Dermatology, University of Massachusetts Medical School, Worcester, MA, USA
*Correspondence: N. Jellinek. E-mail: winenut15@yahoo.com

Abstract
Koilonychia, a concave nail dystrophy, has multiple aetiologies and may be hereditary, acquired or idiopathic. Within der-
matology, koilonychia is often a manifestation of an inflammatory dermatosis such as psoriasis or lichen planus, or a sign
of onychomycosis. Other disease associations include iron store abnormalities, Plummer–Vinson Syndrome, nutritional
deficiencies and occupational or traumatic aetiologies. In young children, koilonychia of the toenails is commonly tran-
sient and idiopathic, although familial and syndromic cases are reported. The dermatologist must be aware of the poten-
tial cutaneous and systemic associations with koilonychia in order to guide appropriate workup, treatment and/or
referral. An algorithm for evaluation of koilonychia is presented along with discussion of common causes of koilonychia
and a comprehensive list of all known associations.
Received: 6 August 2015; Accepted: 4 December 2015

Conflicts of interest
None declared.

Funding sources
None declared.

Introduction tissue, resulting in a relative depression of the distal matrix.3,7,8

Koilonychia describes a transverse and/or longitudinal concave
nail dystrophy where the nail plate is depressed centrally and
everted laterally. The name is derived from the Greek word ‘koi-
los’ which means ‘spoon’.. As the nail first loses its curvature it
may initially appear flat and broad, resulting in platonychia or a
‘petaloid nail.1 The fingernails of the first three digits are prefer-
entially affected, except in early childhood and congenital
cases.2,3 The aetiologies of koilonychia are diverse but can be
divided into hereditary, acquired and idiopathic causes. Most
cases of koilonychias are acquired4 and workup requires a thor-
ough history, review of systems and physical exam.
Pathogenesis
While the pathogenesis of koilonychia is poorly understood, sev-
eral mechanisms have been proposed, including blood flow
abnormalities (age, nervous or vascular changes),5 endocrinopa-
thies, deficiency in metaloenzymes or sulphur-containing amino
acids,6 trauma and primary dermatoses (Fig. 1). One hypothesis
is that poor digital blood flow disrupts the subungual connective
If the distal matrix is depressed in comparison to the proximal
matrix, koilonychia results; whereas, if the distal matrix is higher
than the proximal matrix, clubbing is produced.7,8 Alternatively,
nail bed hyperkeratosis, such as in psoriasis or onychomycosis,
may place pressure on the central distal matrix, producing the
spoon-shaped abnormality (Fig. 2).1
Diagnosis
Koilonychia is a diagnosis made by clinical exam alone. The
finding is more clearly appreciated when the nail is viewed later-
ally. The opposite of pincer nails, spoon nails are flattened in the
middle with everted lateral edges.
Aetiologies and Associations
While the associations with nail spooning are myriad, for the
dermatologist, koilonychia is often seen in the setting of inflam-
matory skin disease, onychomycosis or secondary to anaemia.
An algorithm for evaluation and workup is presented in Fig-
ure 3.

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2 Walker et al.
Figure 1 Mechanisms of Koilonychia. The pathogenesis of koilonychia includes reduced blood flow to the nail, local inflammation or
hyperkeratosis, capillary shunting, endocrinopathies, and/or nutritional deficiencies causing relative depression of the distal matrix and
abnormal nail plate growth.
Figure 2 Koilonychia in psoriasis. Psoriatic nail with koilonychia,
distal nail loss, subungual hyperkeratosis, pitting and focal spotted
lunula.
Dermatologic Associations
Koilonychia is observed frequently in lichen planus (LP) of the
nail, and the true incidence is likely under-represented in the lit-
erature. LP affects the nails in 3–15% of the cases.9,10 In some,
the disease is limited exclusively to the nail apparatus and the
clinical diagnosis can be ambiguous, requiring histopathologic
confirmation. Classic nail findings of LP include nail plate
thinning/atrophy, onychorrhexis, red lunula, polydactylous
longitudinal erythronychia and distal splitting, but koilonychia,
subungual hyperkeratosis and onycholysis may also be observed
(Fig. 4).1,9
The prevalence of nail involvement in patients with psoria-
sis is approximately 80% and is even higher in patient s with
joint disease.11 In up to 5% of patients, nail psoriasis may be
present even in the absence of cutaneous disease.12 The
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Reduction in blood flow
i.e. age, connective tissue disease
Local skin diseases
i.e. psoriasis, lichen planus
Endocrinopathies/
nutritional deficiencies
Capillary “shunt” influences
- Nervous/vascular systems
predominant pattern of nail findings observed depends on
whether the disease targets the nail matrix or the nail bed.
Nail matrix disease is characterized by leuconychia, pitting
and spotted lunula, whereas nail bed disease typically causes
onycholysis, oil-drop spots (salmon patches), a red nail bed
and splinter haemorrhages.12–14 Both patterns may occur
simultaneously. Koilonychia is more common with nail bed
disease.
Trachyonychia, considered to be a better term for 20-nail dys-
trophy as it can affect only one or a few nails, is characterized by
diffuse homogenous nail plate roughness, and may be idiopathic
or associated with alopecia areata, psoriasis, LP or atopic
dermatitis. It is not unusual to observe koilonychia in trachy-
onychia.15
Onychomycosis may present with a spoon-shaped nail defor-
mity, often along with other characteristic findings such as thick-
ened nail, yellow discoloration and subungual debris. The
koilonychia likely results from mechanical subungual pressure
on the nail bed. Additional dermatologic diseases associated with
koilonychia are listed in Table 1.
Systemic Associations
Koilonychia is a classic manifestation of iron store abnormali-
ties, and occurs in 5.4% of the patients with iron deficiency.16
Indeed, some reports claim that iron deficiency is the most com-
mon cause of koilonychia in children.17–19 Nail thinning and
brittleness often accompanies the spoon deformity.5 Severity of
iron deficiency does not appear to correlate with development of
koilonychia, although nail iron levels increase with oral supple-
mentation.16,20 In children, koilonychia may precede laboratory
abnormalities of iron.1,2
© 2016 European Academy of Dermatology and Venereology
Koilonychia: update and review 3

Figure 3 Workup of Koilonychia. Algorithm for the evaluation of koilonychia based on age and associated findings. A thorough history,
review of systems, examination and targeted laboratory workup are necessary for diagnosis and intervention or referral, if appropriate.
PAS, Periodic-Acid Schiff; CBC, complete blood count; TIBC, total iron binding capacity; TSH, thyroid stimulating hormone; CTD,
connective tissue disease; ANA, anti-nuclear antibody.

Plummer–Vinson syndrome (PVS), also known as Paterson– present in 37–50% of the cases, and may be the presenting sign
Kelly syndrome, is a rare entity defined by the triad of dysphagia, of the condition.21,22 Upper aerodigestive tract squamous cell
oesophageal webs and iron deficiency anaemia. Koilonychia is carcinoma occurs in 3–15% of the patients and underlies the

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4 Walker et al.
Figure 4 Koilonychia in lichen planus. Left thumbnail lichen pla-
nus showing koilonychia, onychorrhexis and distal splitting.
importance of making the diagnosis and performing endoscopic
surveillance.23
Koilonychia has been reported in up to 49% of the patients
with hemochromatosis, an autosomal recessive disorder of iron
accumulation (rather than deficiency.) Nail findings may be the
presenting sign of the disease but can occur at any time during
the disease course. Phlebotomy does not appear to normalize
nail plate deformity.24
Koilonychia also occurs in endocrine disorders and is present
in 29% of the cases of hyperthyroidism according to one ser-
ies.25 The nails in hyperthyroid disease tend to be soft and fri-
able, with variable degrees of onycholysis.26 When present in
hypothyroidism, the spoon deformity is accompanied by slow
growth and brittleness.26 Koilonychia in diabetes may be due to
nutrient deficiencies or related to microvascular dysfunction.27
Koilonychia is rarely observed in systemic lupus erythemato-
sus and Raynaud’s disease. The spooning is hypothesized to be
secondary to vasculopathy-induced hypoxia of the nail
matrix.28–30 When present, it may accompany more common
changes such as abnormal proximal nail fold capillary loops,
red lunulae, splinter haemorrhages and nail plate thinning.30,31
Nutritional Associations
Koilonychia may occur in patients with poor nutrition due to
deficiencies in Vitamin C, zinc, copper, selenium, cysteine and
other amino acids.6,32–35 Recent investigations on these associa-
tions are lacking. Studies of populations predisposed to poor
nutrition (such as child labourers, rural villagers, alcoholics and
patients with chronic kidney disease) document koilonychia in
5.5–18%.33,36–39
Congenital and Early Childhood Koilonychias
The five most common nail findings in otherwise normal
children are: punctate leuconychia, sequelae of onychopha-
gia, pitting, koilonychia (especially of the big toe) and
lamellar splitting of the free edge (often associated with
koilonychia).
Transient acquired koilonychia is observed in at least 5% of
normal 2-year olds.15 In a review of 224 children in Israel, 27%
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had idiopathic koilonychia of the toes, and the rates were higher
in communities where children walked barefoot. Toenail
koilonychia was most common at 1–2 years of age and was
rarely present after the age of 9.40
While isolated koilonychia of the toenails in children is usu-
ally idiopathic, it remains a diagnosis of exclusion, and anaemia
and nutritional deficiencies should be considered.2,3 Trauma is a
common cause of koilonychia in children, often due to tightly
fitting shoes or thumb/finger sucking. Nail growth normalizes
with behaviour modification.4
Familial koilonychia, while rare, has been appreciated in
several pedigrees and is inherited in an autosomal domi-
nant fashion with a high degree of penetrance and no
predilection for sex.4,41–47 It may present at birth or within
the first few years of life. Nails are typically thin and flat,
developing degrees of concavity over time. Variable expres-
sion is noted, with some family members having only fin-
gernails affected, or involvement of only the fingernails on
one hand, on select fingers or toes, or with accentuation of
the thumb and/or great toe.43,44 Rarely, koilonychia may be
seen as a part of a genodermatosis (Table 1).
Regional
An increased prevalence of koilonychia has been observed in
high-altitude living conditions (>3000 metres above sea level),
with an incidence of 7–47% reported in certain populations in
India, in the absence of iron deficiency.48–53 Koilonychia usu-
ally presents in the fourth or fifth decade, after several years of
high-altitude habitation. Altitude-associated haematologic
changes, vegetarian diets with low iron content, manual labour
(trauma) and the high silica content of the soil are speculated
to contribute to koilonychia in these communities.52
Occupational
Koilonychia may also be related to the patient’s occupation,
either from exposure to an irritant and/or mechanical stress on
the digit; over time, these changes may become irreversible.
Dawber et al. documented fingernail koilonychia in 5.3% of car
mechanics as opposed to none in their control group.54 Similar
findings have been noted in other occupations with exposure to
mineral oil, organic solvents and chemicals causing contact der-
matitis.55–57 Hairdressers who give permanent waves with
ammonium thioglycolate describe painful fingertips and spoon-
ing of the nails over time, without associated dermatitis.58
Repetitive trauma alone has led to koilonychia in a variety of
other occupations.58,59
Workup and Treatment
The aetiologies of koilonychia are numerous but the underlying
diagnosis can be narrowed based on age, personal, family and
occupational history, review of systems and a thorough physical
exam. We propose an algorithm to guide clinical evaluation
© 2016 European Academy of Dermatology and Venereology
Koilonychia: update and review 5

Table 1 Classification of Koilonychia (adapted from Baran’s and Stone OJ)1,8

I. Idiopathic forms III. Acquired forms

II. Hereditary and congenital forms
• Genodermatoses
Fissured nails, in adenoma sebaceum
Monilethrix63
Hereditary osteo-onychodysplasia
(Nail-patella syndrome)
Nezelof’s syndrome (immunological defect)
Witkop’s tooth and nail syndrome64
Oliver-McFerlane syndrome
(Congenital Trichomegaly)65
Kindler’s syndrome66
Trichothiodystrophy67
Trichorhinophalangeal syndrome68
Neonatal ichthyosis-sclerosing cholangitis (NISCH)69
Leuconychia and sebaceous cysts70,71
Palmoplantar keratoderma (Meleda type)8
Incontinentia pigmenti8
Dermatologic
• Lichen planus, Psoriasis,4,13,14 Trachyonychia,15 Porphyria cutanea tarda
• Scleroderma, Raynaud’s disease8
• Systemic lupus erythematosus, Acanthosis nigricans, Alopecia areata
• Cronkhite-Canada syndrome, Darier’s disease
Cardiovascular and Haematological
• Iron deficiency anaemia
• Iron loss, bleeding, nutritional deficiencies (i.e. celiac disease,23,79 peptic ulcer disease,23 helminth
infection, inflammatory bowel disease,22 gastrointestinal bleeding,23 menorrhagia,23,80 pregnancy,81
poor nutrition17)
• Plummer-Vinson syndrome
• Iron malabsorption
• Sickle cell disease82
• Pyruvate kinase deficiency
• Polycythemia vera83

Palmar hyperkeratosis8 • Hemochromatosis24

LEOPARD syndrome8 • Banti’s syndrome/portal hypertensive biliopathy
Gottron’s syndrome (acrogeria)8 • Coronary disease
Ectodermal dysplasia with anhidrosis8 • Primary amyloid8
Chondroectodermal dysplasia Infectious disease
(Ellis-van Creveld syndrome)8 • Fungal diseases (onychomycosis)
Focal dermal hyperplasia (Goltz syndrome)8 • Syphilis
• Isolated congenital dysplasia with • Scabies
longitudinal angular ridging72 Endocrine
• Non-Familial twenty nail dystrophy73 • Diabetes
• Congenital koilonychia associated with • Hypothyroidism
dome-shaped epiphyses and • Hyperthyroidism, Thyrotoxicosis32
vertebral platyspondia74 • Acromegaly
• Familial – autosomal dominant Traumatic and occupational
Familial koilonychia with keratosis pilaris75 • Petrol, solvents, engine oils (mechanics,54 cabinet-makers,55 homemakers, railway workers,57
Familial koilonychia with syndermatotic cataract76 oil-burner repairers84)
Familial severe twenty nail dystrophy77 • Acids, alkalis (cement workers/ brick-layers),56 thioglycolate (hairdressers),58 sulphur and arsenic
• Foetal exposure to polychlorinated (chemical hair removal products)85
biphenyl (PCB)78 • Mechanical trauma (rickshaw boys,59 butchers,84 mushroom growers, coil winders, slaughterhouse
• Idiopathic age-associated, particularly workers,84 pin threaders)
in big toes in early childhood • Thermal burns8
• Nail biting, thumb/finger sucking, poorly fitting shoes
• High altitude ‘Ladakhi koilonychia’26,49–52,54
• Avitaminosis/Nutritional
• Nutrients
• Protein deficiency/low albumin32
• Vitamin C13
• Vitamin B1213
• Niacin, zinc, copper
• Sulphur-containing amino acids: methionine, cystine
• Populations
• Kidney transplantation, End stage renal disease34,39
• Alcoholism33,38
• Poor nutrition, cachexia36,37
• Post-gastrectomy8
Liver Cell Adenoma
Erythropoietin-producing tumours8
Carpal Tunnel Syndrome86

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6 Walker et al.
(Fig. 3). Appropriate workup is important to exclude an under-
lying cutaneous or systemic disorder.
Lichen planus of the nail can be scarring, leading to perma-
nent loss of the nail matrix with dorsal pterygium and anony-
chia. Therefore, early diagnosis and discussion of the treatment
options (though limited) is important. The potential for a medi-
cation-induced lichenoid reaction should be considered60 and a
thorough examination performed to exclude mucocutaneous
involvement. Nail LP may improve with a prolonged course of
systemic steroids, but relapses are common.61 Nail psoriasis may
be treated with a variety of therapeutics including topical steroid
or calcipotriene, intralesional steroid injections and systemic
agents according to the site of the pathology. Again, potential
medication causes of drug-induced psoriasiform dermatitis
should be considered .
Nails in iron deficiency anaemia usually return to normal
within 4–6 months of iron repletion. PVS is treated with iron
supplementation and mechanical dilation of oesophageal webs.
Rapid improvement in symptoms of dysphagia and fatigue may
occur within weeks of iron therapy.62 Patients should be
screened for aerodigestive tract carcinoma. All other treatments
of koilonychia are directed at the underlying aetiology.
Conclusion
Koilonychia can be an important clue in a dermatologic or sys-
temic condition. Careful evaluation of other nail and skin find-
ings as well as review of systems can help the clinician narrow
their differential diagnosis. In acquired forms, most cases are
reversible.
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