Novel Insights from Clinical Practice

Skin Appendage Disord 2018;4:326–330 Received: November 21, 2017

Accepted: November 28, 2017
DOI: 10.1159/000485854 Published online: January 10, 2018

Onychogryphosis: Case Report and

Review of the Literature
Dayoung Ko a Shari R. Lipner b
   

a Duke
University School of Medicine, Durham, NC, USA; b Department of Dermatology, Weill Cornell Medicine,
 

New York, NY, USA

Established Facts

• Onychogryphosis is a common nail condition seen in older individuals and patients with poor per-
sonal care.
• If left untreated, it may cause pain, paronychia, or onychocryptosis and is often a source of embarrassment.

Novel Insights

• Onychogryphosis may be managed conservatively by limiting pressure on the nail bed and using me-
chanical debridement.
• Definitive treatment for onychogryphosis is nail avulsion followed by matricectomy or surgical resec-
tion of the nail matrix.

Keywords yellow discoloration of his left great toenail, clinically consis-

Nails · Onychogryphosis · Nail avulsion · Matricectomy · tent with onychogryphosis. This review highlights the etiol-
Phenolization ogy, diagnosis, and treatment options for onychogryphosis.
© 2018 S. Karger AG, Basel

Abstract
Onychogryphosis is a disorder of nail plate growth that is Introduction
clinically characterized by an opaque, yellow-brown thick-
ening of the nail plate with associated gross hyperkeratosis, Onychogryphosis is a disorder of nail plate growth that
elongation, and increased curvature. It is most common in is characterized by an opaque, yellow-brown thickening
older adults and patients with poor personal care. It is a rela- of the nail plate with associated gross hyperkeratosis,
tively common and important condition because patients elongation, and increased curvature. It is often described
may suffer from pain, secondary complications, and cosmet- as a “ram’s horn nail” or being “oyster-like” in appear-
ic concerns. We present the case of a 67-year-old man with a ance. The nail plate initially grows upwards and thereafter
30-year history of severe thickening, abnormal growth, and deviates laterally towards the other toes [1, 2]. According

© 2018 S. Karger AG, Basel Shari R. Lipner, MD, PhD

1305 York Avenue
New York, NY 10021 (USA)
E-Mail [email protected]
E-Mail shl9032 @ med.cornell.edu
www.karger.com/sad
 Discussion

Color version available online

Prevalence
The prevalence of onychogryphosis is highest in the
elderly population. In one cross-sectional observational
study on 173 patients in 3 nursing homes in Tokyo, the
prevalence of onychogryphosis was 17.9% [5]. In another
study conducted on 536 patients over the age of 75 years
from 4 general practices, the reported prevalence was
11.2% [6]. In a hospital survey conducted on foot prob-
lems in 100 patients in 4 different wards, the reported
prevalence was 38% [7].

Causes
Congenital Onychogryphosis
There are few reports of inherited cases of onychogry-
phosis. Congenital onychogryphosis has been described
and inherited as an autosomal-dominant trait in which all
Fig. 1. Onychogryphosis of the left great toenail. Thickening and the nails of both hands and feet may be involved. Onset is
lateral deviation of the left first toenail, resembling a “ram’s horn.” usually within the first year of life [8]. A case of congeni-
tal onychogryphosis was described in a 2-year-old girl
who was born prematurely with anonychia. When all the
nails grew, they were hypertrophic and deviated laterally
[9]. Congenital onychogryphosis may clinically resemble
to Zaias [3], the nail plate grows hypertrophied and un- and must be distinguished from congenital malalignment
evenly at the matrix, and the faster growing side is what of the toenails and congenital pachyonychia [10, 11]. On-
determines the direction of the deformity. Two possible ychogryphosis is also a feature of Haim-Munk syndrome
explanations for this deformity have been proposed: there along with palmoplantar hyperkeratosis, severe early-on-
may be insufficient nail matrix under the posterior fold to set periodontitis, pes planus, arachnodactyly, and acro-
exert a flattening effect, or the nail bed may contribute a osteolysis. Haim-Munk syndrome is an autosomal reces-
greater quantity of keratin to the nail than is normally sive genodermatosis caused by mutations in a lysosomal
seen [4]. The great toenail is most often affected, but the protease gene, cathepsin C [12]. Onychogryphosis has
fingernail may be involved in rare cases. It is more com- also been rarely reported to be associated with tuberous
monly seen in elderly or homeless patients with signs of sclerosis, which may be due to poor nursing care, cogni-
neglect [1]. tive impairment, or subungual fibromas [13]. There have
been reports of onychogryphosis as a feature of several
congenital syndromes, including Papillon-Lefèvre syn-
Case Report drome [14], ichthyosis hystrix [15], and ectodermal dys-
plasia-syndactyly syndrome type I [16].
A 67-year-old man presented with a 30-year history of severe
thickening, abnormal growth, and yellow discoloration of his left Acquired Onychogryphosis
great toenail. He stated that the affected nail broke off midway ev- Acquired onychogryphosis is most often observed in
ery 6 months and then regrew. It had become more painful re-
cently and was impinging on his second toe, making it difficult to people with long-standing poor personal care or neglect,
walk and put on socks. There was no history of trauma, tinea pedis, in homeless persons, and in people with senile dementia.
or family history of onychomycosis. Physical examination was no- Onychogryphosis may be associated with ichthyosis, pso-
table for thickening and lateral deviation of the left first toenail riasis, pemphigus, syphilis, variola, and hyperuricemia [1,
(Fig. 1). A nail clipping with histopathology was negative for fun- 17, 18]. Onychogryphosis is also seen with impairment of
gal hyphae. An X-ray of the toe showed mild osteoarthritis, and
there was no subungual exostosis. A diagnosis of onychogryphosis the peripheral circulation, including varicose veins, stasis
was made, and a nail avulsion with phenol matricectomy was per- dermatitis, and ulcers of the lower legs [1, 18, 19]. Ony-
formed as therapy. chogryphosis has been reported to be a result of an elastic

Onychogryphosis Skin Appendage Disord 2018;4:326–330 327

DOI: 10.1159/000485854
wire for correction of a pincer nail [20]. Traumatic inju- Table 1. Causes of acquired onychogryphosis
ries to the nails, including burn injuries and microtrauma
Causes of acquired onychogryphosis
resulting in suppuration or scarring, may also result in
onychogryphosis [4]. Microtrauma is often attributed to Poor self-care Advancing age
improperly fitting shoes or footgear, and foot anomalies, Homelessness
such as hallux valgus, may cause the nail to be more sus- Senile dementia
ceptible to trauma [18, 21]. Table 1 summarizes causes of Dermatologic diseases Ichthyosis
acquired onychogryphosis. Pemphigus
Interestingly, onychogryphosis and lymphadenopathy Psoriasis
are present in 50–75% of dogs infected with leishmaniasis Infectious diseases Onychomycosis
[22, 23]. However, to date, there have been no reports of Syphilis
humans with leishmaniasis having onychogryphosis [24]. Variola
Local causes Major trauma to the nail
Diagnosis Microtrauma caused by footwear
Onychogryphosis remains largely a clinical diagnosis Foot anomalies (e.g., hallux valgus)
based on characteristic appearance. There is thickening Nail surgery
of the nail plate with associated gross hyperkeratosis and Medical disorders Varicose veins
increased curvature. The nail can be oyster-like or resem- Thrombophlebitis
ble a ram’s horn, and its irregular surface is marked by Ulcers of the lower leg
Hyperuricemia
longitudinal and transverse striations, the latter of which
are more frequent. Diagnosis of onychogryphosis can be
difficult to reach in the early stages because hypertrophy
of the nail plate is the earliest manifestation, with the
more classical features appearing later [1]. Histologically, should be made to avoid excessive pressure on the nail
there is a disorderly arrangement of keratinocytes within bed. Footwear should be reviewed to assess appropriate
the nail plate with hyperchromatism, parakeratosis, and fit, avoiding excessive pressure or microtrauma to the
numerous splits [25]. The disorder may be confused clin- toes [25]. Conservative methods are preferred in the el-
ically with onychomycosis, and fungal studies can be used derly population, particularly in patients with vascular
to rule out the latter. It should be noted, however, that impairment or hyperglycemia due to diabetes. In the ma-
onychomycosis and onychogryphosis can coexist. jority of these cases, regular use of electric drills, burs, or
Hemi-onychogryphosis is a condition mimicking on- mechanical debridement with a dual-action nail nipper
ychogryphosis in which the nail plate grows laterally from (Fig.  2) will help establish and maintain a reduction of
birth or early childhood. This may be a complication of nail plate thickness and length [27, 28]. Using cryothera-
persistent congenital malalignment of the great toenails. py on the nails before nail trimming makes it easier to cut
It can be prevented by regular nail plate trimming and the nail with an even edge [29].
foot care [2]. In patients with good vascular supply, a nail avulsion
or removal of the thickened nail plate may be indicated.
Complications A blunt dissection using a regular nail clipper can be used
Some common complications of onychogryphosis in- after chemical avulsion with 40% urea or 50% potassium
clude ingrown toenails, paronychia, secondary onycho- iodine under occlusion. Onychogryphosis invariably re-
mycosis, and the inability to cut the toenail due to increas- curs after a simple nail avulsion [30]. In 1 study, a simple
ing hypertrophy of the nail plate [21]. Rarely, the lateral nail avulsion was performed on 29 onychogryphosis pa-
part of the proximal nail fold may become indurated [26]. tients who had failed conservative treatment. The recur-
Infrequently, subungual gangrene may ensue [2]. rence rate after 12 months was 93%; however, only 17%
were symptomatic [31].
Management and Treatment If conservative methods fail and the patient is symp-
Treatment is warranted to prevent complications and tomatic, the definitive treatment is nail avulsion followed
for cosmesis [2] (Table 2). Therapy depends on the cause by matricectomy. In 1 study conducted on 96 patients
and patient comorbidities; onychogryphosis can be treat- with recurrent onychogryphosis, the authors reported a
ed palliatively or operatively [21]. Initially, every effort significant advantage of the nail avulsion with matricec-

328 Skin Appendage Disord 2018;4:326–330 Ko/Lipner

DOI: 10.1159/000485854
 Table 2. Treatment options for onychogryphosis

Therapy Methods

Conservative treatment (nonsurgical) Dual-action nail nippers, mechanical burs, and drills for
mechanical debridement of the nail
Simple nail avulsion without matricectomy Blunt dissection after chemical avulsion
Nail avulsion with matricectomy Chemical ablation (phenolization)
Cold steel
Zadik procedure
Electrosurgery
CO2 laser
Nail avulsion with matricectomy with V-Y Triangular flap designed with the apex at the distal
advancement flap interphalangeal crease and the base at the edge of the lesion
Syme method One-half of the terminal phalanx is excised together with the
nail fold

control and the Zadik technique, 2 oblique 0.5-inch inci-

Color version available online

sions are made, beginning at the junction of the nail fold

and nail walls, extending laterally and proximally [34].
Sharp dissection is used to avulse the nail and remove the
germinal matrix and the overlying deep layer of the nail
fold and walls. The skin flaps are sutured back to cover
the wound [30]. This technique can also be modified to
be a partial matricectomy, in which an entire wedge of
nail matrix is excised [33]. Another surgical technique
utilizes a matricectomy with a V-Y advancement flap,
which is a triangular flap designed with the apex at the
distal interphalangeal crease and the base at the edge of
the lesion. The advantages of the V-Y advancement flap
are preservation of sensation and length and good soft-
tissue coverage [35]. The most radical technique, rarely
used today, is the Syme method, in which one-half of the
terminal phalanx is also excised together with the nail fold
[36].
Fig. 2. Dual-action nail clippers. This device can be used for diag-
nostic and therapeutic purposes for onychogryphosis. Ablation of the nail matrix can be performed with elec-
trodessication. Advantages of this method are minimal
postoperative pain and drainage and that it can be per-
formed quickly. Ablation may also be performed by che-
mocauterization with phenol (supersaturated solution,
tomy compared to a simple nail avulsion alone [32]. Ma- 88%) or with 10% sodium hydroxide with excellent success
tricectomy can be achieved either by excision or ablation rates [9, 37]. In a study of 107 patients with onychogrypho-
[33]. Excisional techniques include cold steel, cutting sis or ingrown toenails, phenol cauterization was superior
electrosurgery, or a laser used in the cutting mode. Abla- to Zadik procedure for both nail conditions. Phenol cau-
tive techniques include chemocautery, electrocautery, or terization resulted in fewer recurrences of nail spikes as
a laser in the ablative mode [33]. well as fewer cases requiring further surgical treatment
In some cases, surgical resection of the entire nail ma- [37]. The carbon dioxide (CO2) laser is also used to treat
trix is indicated. Using local anesthesia and tourniquet onychogryphosis [38]. Advantages of this technique in-

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DOI: 10.1159/000485854
clude more selective destruction of the matrix epithelium method for the treatment of onychogryphosis, while min-
without causing necrosis of adjacent dermal tissue, less imizing adverse effects.
postoperative pain, and shortened healing time [38].

Statement of Ethics
Summary
The patient’s consent has been obtained for the publication of
Figure 1.
Onychogryphosis is an important nail condition to
recognize and treat due to pain and sequelae, including
onychocryptosis and paronychia, and for cosmesis. De-
Disclosure Statement
pending on the patient’s comorbidities, onychogryphosis
can be managed conservatively or surgically. Further Dayoung Ko and Dr. Lipner have no conflicts of interest to dis-
studies are needed to evaluate the most beneficial surgical close.

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DOI: 10.1159/000485854