3910 Chin Med J 2013;126 (20)

Original article
A new classification of nevus of Ota
HUANG Wen-hui, WANG Hong-wei, SUN Qiu-ning, JIN Hong-zhong, LIU Yue-hua, MA Dong-lai, ZUO Ya-gang,
ZHENG He-yi, WAN Kuo, JING Quan and ZHAO Yong-liang

Keywords: nevus of Ota; classification; trigeminal nerve

Background The nevus of Ota, is a common benign pigmentary dermatosis, mainly involve innervation area of first and
second branch of trigeminal nerve. The classification of nevus of Ota was proposed by Tanino, based on 26 cases of
nevus of Ota from 1937 to 1940. Studies about its classification are rarely seen in last 70 years, while it is still practical
today.
Methods Based on the clinical photographs, 1079 consecutive patients with nevus of Ota were verified and reclassified
according to the innervation areas of the trigeminal nerve branches.
Downloaded from http://journals.lww.com/cmj by BhDMf5ePHKbH4TTImqenVChdHhRBUo+1jKcvULv0b3x72y7WOyLiyBSClRZAM97b on 10/08/2020

Results In these 1079 cases, 866 patients were in line with Tanino’s classification (80.26%), and 213 patients were not
(19.74%). We put forward a new clinical classification (Peking Union Medical College Hospital classification, PUMCH
classification) of nevus of Ota based on the innervation area of the trigeminal nerve branches, composed of 5 types and
14 subtypes. The 5 types were as follows: Type I — pigmentation maculeses involving the innervation area of one of the
three trigeminal nerve branches, of which there were 424 cases (39.3%), comprising 6 subtypes; Type II — pigmentation
macules involving the innervation area of two branches of the three trigeminal nerve branches, of which there were 221
cases (20.48%), comprising 4 subtypes; Type III — pigmentation macules involving the innervation area of all three
trigeminal nerve branches, of which there were 361 cases (33.45%), comprising 2 subtypes; Type IV — bilateral type,
in which the pigmentation macules involves the bilateral cheek, of which there were 63 cases (5.84%), comprising 2
subtypes; and Type V — complications occurred in the patient, of which there were 10 cases (0.93%).
Conclusion The new classification of nevus of Ota is based on the innervation area of the trigeminal nerve branches, and
it covers all types of Tanino’s classifications; on that basis, some new types and subtypes are brought in and cover almost
every clinical condition.
Chin Med J 2013;126 (20): 3910-3914

N evus of Ota, also known as nevus fuscocaeruleus

ophthalmo-maxillaris, first reported by Ota and
Tanino in 1939, 1,2 is a benign pigmentary dermatosis
which involves innervated areas of the first branch (V1)
and second branch (V2) of the trigeminal nerve.3 It mainly
affects the eye region and partes zygomatica, and the color
of the skin lesion is brown or blue, the diameter of the area
is 1–10 cm or larger. At present, it is believed that nevus
of Ota is caused by heteroplasia that occurs in melanocyte
migration during embryonic development. Tanino4 analyzed
and classified 26 patients with nevus of Ota. In Tanino’s
classification, the nevus of Ota was divided into 4 types
according to skin involvement area. This classification
has been used for more than 70 years. After the study,
follow-up and the sum-up of all cases of nevus of Ota in
the last 12 years, the Dermatology Department of Peking
Union Medical College Hospital (PUMCH) proposed a
new classification based on the innervations area of the
Trigeminal nerve branches.
METHODS
Study population
A total of 1139 patients with nevus of Ota were from the
Department of Dermatology, PUMCH between January
1998 and June 2010. Of these 1139 patients, 1079 had
complete clinical records to analyze – 206 males and 873
females. The sex ratio of male to female was 1:4.24. Age
ranged from 4 months to 69 years old, with an average age
of 22.79 years.
Investigative method
The investigative methods included interrogation enquiry,
dermatological examination, reviewing clinical records,
checking patient’s morphological appearance and making
phone calls and so on. The results of the investigation were
recorded on forms. The study was approved by the local
ethics committee and all patients singed informed consent
prior to participation.
Tanino’s classification of nevus of Ota
A total of 1079 patients were checked and verified
DOI: 10.3760/cma.j.issn.0366-6999.20131211
Department of Dermatology (Huang WH, Wang HW, Sun QN, Jin
HZ, Liu YH, Ma DL, Zuo YG, Zheng HY and Zhao YL), Department
of Stomatology (Wan K and Jing Q), Peking Union Medical College
Hospital, Chinese Academy of Medical Sciences, Beijing 100730,
China
Correspondence to: Prof. WANG Hong-wei, Department of
Dermatology, Peking Union Medical College Hospital, Chinese
Academy of Medical Sciences, Beijing 100730, China (Tel: 86-10-
69156387. Fax: 86-10-58205247. Email: whwtyw@263.net)
The authors have no conflicts of interest to declare.
Chinese Medical Journal 2013;126 (20)
according to Tanino’s classification: 4 nevus of Ota
was divided into 4 types according to the skin lesion
involvement area: Type I was mild, the pigmentation
macules area was limited, and it can be further subdivided
into four categories: Type Ia – eye region type, the
pigmentation macules involved skin of the upper and lower
eyelid, orbit, and part of the temporal region; Type Ib –
zygomatic region type, the pigmentation macules involved
the skin of the lower eyelid and zygomatic region; Type
Ic – forehead type; and Type Id – nostril type. Type II was
moderate, and the pigmentation macules involved skin of
the upper and lower eyelid, orbit, zygomatic region, cheek,
temporal region, nasion, and alae of nose. Type III was
severe, and the pigmentation macules involved the scalp,
forehead, upper and lower eyelid, orbit, zygomatic region,
cheek, temporal region, nasion, alae of nose, and posterior
and superior area of auricle. Type IV was bilateral type.
Innervations area of trigeminal nerve branches
The innervations area of trigeminal nerve branches5 were
shown in Figure 1.
The first branch-ophthalmic nerve: the lacrimal nerve
innervates the upper eyelid; the frontal nerve innervates
the skin of forehead and calvaria; the nasociliary nerve
innervates the dorsum nasi.
The second branch-maxillary nerve: the infraorbital nerve
innervates the skin of the lower eyelid, the alae of the nose,
the upper lip; and the zygomaticus nerve innvervates the
skin of zygomatic region and the temporal region.
The third branch-mandibular nerve: the buccal nerve
innvervates the skin of the cheek; the auriculo-temporal
nerve innervates the skin of the temporal region and scalp;
the mental nerve innervates skin of the mentum and lower
lip.
RESULTS
A total of 866 patients were in accordance with Tanino’s
classification (80.26%), in which there were 442 cases
of Type I (40.96%), 185 cases of Type II (17.15%),
176 of Type III (16.36%), and 63 of Type IV (5.9%).
The remaining 213 cases were not in line with Tanino’s
Figure 1. Innervations area of trigeminal nerve branches. I:
Innervations of ophthalmic nerve; II: Innervations of maxillary
nerve; III: Innervations of mandibular nerve.
3911
classification (19.74%). In those 442 Type I cases that were
in line with Tanino’s classification, however, there were 46
cases of Type Ia (4.26%), 380 cases of Type Ib (35.22%),
11 of Type Ic (1.02%), and 5 of Type Id (0.46%) (Table 1).
There is no detailed description of the symmetry of bilateral
skin involvement in Type IV of Tanino’s classification.
We observed the above-mentioned 63 cases of Type IV,
and in 36 of these patients, the bilateral pigmentation
macules were completely symmetrical, including 2 cases of
pigmentation macules located on the tip of nose, which also
should be classified into Type IV (Figure 2). The remaining
27 cases were asymmetric.
A total of 213 cases were not in line with Tanino’s
classification. The clinical manifestation of these 213
patients was reported as follow (Figure 3): A: 25 cases
had pigmentation macules on the skin of the forehead and
upper eyelid, i.e. innervation areas of ophthalmic nerve
branches – frontal nerve and lacrimal nerve; B: 3 cases had
pigmentation macules involved the cheek, i.e. innervation
area of third trigeminal nerve branch – buccal nerve; C: 60
cases had pigmentation macules on the skin of the orbit,
zygomatic region, and temporal region, i.e. innervations
area of ophthalmic nerve branches – lacrimal nerve, as well
as innervation area of maxillary nerve branch – infraorbital
nerve, zygomatic nerve; D: 49 cases had pigmentation
macules on the skin of the forehead, orbit, zygomatic
region, and temporal region, i.e. innervation areas of
ophthalmic nerve branches, frontal nerve, lacrimal nerve,
as well as innervation areas of maxillary nerve branch
– infraorbital nerve, zygomatic nerve; E: 66 cases had
pigmentation macules on the skin of the zygomatic region,
temporal region, cheek, and anterior auricle; F: 10 cases
had complications, including 5 cases that combined Port-
Wine Stains (PWS), 4 cases that combined telangiectasia,
and 1 case that combined vitiligo.
After analyzing the 213 cases that were not in line with
Tanino’s classification, we found that the pigmentation
macules distribution can be classified according to the
innervation areas of the trigeminal nerve branches; so we
suggest that nevus of Ota be reclassified according to the
innervation area of the trigeminal nerve branches as below,
named PUMCH classification (Figure 4).
Type I: pigmentation macules involving one branch of the
trigeminal nerve. Type Ia: pigmentation macules involving
innervation area of first branch (ophthalmic nerve) of
Table 1. Proportion of each type in 1079 patients of nevus of Ota
Types n (%)
Tanino type I
Ia 46 (4.26)
Ib 380 (35.22)
Ic 11 (1.02)
Id 5 (0.46)
Tanino type II 185 (17.15)
Tanino type III 176 (16.31)
Tanino type IV 63 (5.84)
Not in line with Tanino’s classification 213 (19.74)
3912
Figure 4. PUMCH classification of nevus of Ota. A: Type Ia1; B: Type Ia2; C: Type Ib1; D: Type Ib2; E: Type Ib3; F: Type Ic; G: Type
IIa1; H: Type IIa2; I: Type IIa3; J: Type IIb; K: Type IIIa; L: Type IIIb.
trigeminal nerve; Type Ia1: pigmentation macules involving
forehead, i.e. innervation area of frontal nerve; Type Ia2:
pigmentation patch involving forehead and eyelid, i.e.
innervation area of frontal nerve and lacrimal nerve; Type
Ib: pigmentation macules involving innervation area of
second branch (maxillary nerve, V2) of trigeminal nerve;
Type Ib1: pigmentation macules involving lower eyelid
and inferior area of lower eyelid, i.e. innervation area
of infraorbital nerve; Type Ib2: pigmentation macules
involving lower eyelid, zygomatic region, temporal region,
i.e. innervation area of infraorbital and zygomatic nerves;
Type Ib3: pigmentation macules involving skin of alae of
nose, i.e. innervation area of infraorbital nerve; Type Ic:
pigmentation macules involving innervation area of third
branch (mandibular nerve, V3) of trigeminal nerve. There
are 3 patients whose pigmentation macules involved the
skin of the cheek, which is the innervation area of the
buccal nerve.
Type II: pigmentation macules involving two branches
of the trigeminal nerve. Type IIa: pigmentation macules
involving the first and second branches (ophthalmic and
maxillary nerves; V1 + V2) of the trigeminal nerve; Type
Chin Med J 2013;126 (20)
Figure 2. The pigmentation macules involve tip of nose.
Figure 3. Special manifestations are not in line with
Tanino’s classification. 3A: Innervation areas of frontal
nerve, lacrimal nerve; 3B: Innervation areas of buccal
nerve; 3C: Innervation areas of lacrimal nerve, infraorbital
nerve, zygomatic nerve; 3D: Innervation areas of frontal
nerve, lacrimal nerve, infraorbital nerve, zygomatic nerve;
3E: Innervation areas of trigeminal nerve V2 and V3.
IIa1: pigmentation macules involve orbit, i.e. innervation
area of lacrimal and infraorbital nerves; Type IIa2:
pigmentation macules involving orbit, zygomatic region,
temporal region, i.e. innervation area of infraorbital and
zygomatic nerves; Type IIa3: pigmentation macules
involving forehead, orbit, zygomatic region, and temporal
region, i.e. innervation area of lacrimal, infraorbital
and zygomatic nerves; Type IIb: pigmentation macules
involving skin of the zygomatic region, temporal region,
cheek, anterior auricle, i.e. innervation area (maxillary
nerve and mandibular nerve; V2 + V3) of the second and
third branch of trigeminal nerve.
Type III: pigmentation macules involving the first,
second and third branches (ophthalmic, maxillary, and
mandibular nerves; V1 + V2 + V3) of the trigeminal
nerve. Type IIIa: pigmentation macules involving skin of
orbit, zygomatic region, temporal region, cheek, anterior
auricle, i.e. innervation area of the first, second and
third branches (ophthalmic, maxillary, and mandibular
nerves; V1 + V2 + V3) of the trigeminal nerve; Type IIIb:
pigmentation macules involving skin of forehead, orbit,
zygomatic region, temporal region, cheek, anterior auricle,
Chinese Medical Journal 2013;126 (20) 3913

i.e. innervation area of the first, second and third branch Clinical classification is very important to define the
(ophthalmic, maxillary, and mandibular nerves; V1 + V2 + extent of a disease. Because of the complex and diverse
V3) of the trigeminal nerve. manifestation of nevus of Ota, a practical, highly
generalized classification system is needed in clinical
Type IV (bilateral type): bilateral pigmentation maculeses work. In recent years, clinical study on nevus of Ota is
respectively involve innervation areas of one or more mainly focused on the treatment of the disease10-13 but the
branches of the trigeminal nerve. Type IVa (symmetric): the clinical classification reports of nevus of Ota is rare in
pigmentation maculeses are symmetrical. Two patients in the literature. Tanino’s classification takes the severity of
this group had pigmentation maculeses that involved the tip disease into account, and gives each type a diagrammatic
of nose. They are type IV too; Type IVb (asymmetric): the presentation (e.g., Type I). It covers various categories. In
pigmentation macules are asymmetrical. our study, the compliance rate is up to 80.26%. However,
Tanino’s classification has several limitations: its sample
Type V: nevus of Ota combined complications: 10 cases in size is small (26 cases); it cannot cover all types of clinical
this group had complications, including 5 cases combined manifestations; the classification of the bilateral type
with Port-Wine Stains (PWS), 4 cases combined with is too simple; the symmetry of pigmentation macules
telangiectasia, and 1 case combined with vitiligo. involvement was not mentioned; and the complications
of nevus of Ota were not considered. In our study, we
The proportion of each type in PUMCH classification is appended the situation about the severity of bilateral type
shown in Table 2. The comparison between the PUMCH and whether there is complication into old classification,
classification and Tanino’s classification is shown in Table added manifestation of special region into it, divided types
3. into subtypes, and increased the amount of clinical types to
cover as many of the clinical manifestations as possible.
DISCUSSION
Over the years, only a few scholars have tried to reclassify
Nevus of Ota is a frequently benign, congenital and nevus of Ota according to a new standard. In 1991,
unilateral melanocytic pigmentary disorder, generally Hirayama and Suzuki14 conducted a pathological analysis of
involving the area innervated by the first and second 450 patients of nevus of Ota, classifying nevus of Ota into 5
divisions of the trigeminal nerve.6,7 It is more common in types according to the melanocyte distribution in the derma.
Asians and African descent, but very rare in Caucasians.8,9 In other words, there were pathological types of nevus
of Ota. Since most patients do not need to be confirmed
by pathological diagnosis, this classification is limited in
Table 2. Proportion of each type in PUMCH classification of nevus
of Ota of 1079 study patients
value. In 2001, Chan et al15 analyzed 119 patients of nevus
PUMCH classification n (%) of Ota who accepted laser therapy, and he classified nevus
Type I of Ota into 4 types. However, since this classification lacks
Ia1 11 (1.01) morphological description, it is unable to reflect distribution
Ia2 25 (2.32)
and area of the pigmentation macules.
Ib1 97 (8.99)
Ib2 283 (26.23)
Ib3 5 (0.46) A modified classification system that indicates the extent of
Ic 3 (0.28) nevus of Ota is needed. The PUMCH classification of nevus
Type II of Ota is based on a large number of patients’ data from
IIa1 46 (4.26)
PUMCH over the last 12 years; we photographed these
IIa2 60 (5.56)
IIa3 49 (4.54)
patients, collected their clinical information, followed up,
IIb 66 (6.12) analyzed and concluded all of these cases. After the study,
Type III we reclassified nevus of Ota into 5 types and 14 subtypes
IIIa 185 (17.15) according to innervation area of the trigeminal nerve
IIIb 176 (16.31)
branches. Of these cases, 80.26% were in compliance with
Type IV
IVa 36 (3.34)
both Tanino’s classification and PUMCH classification;
IVb 27 (2.50) 19.74% were in line with the new classification. The
Type V 10 (0.93) PUMCH classification covers all common manifestations
of nevus of Ota, as well all types of Tanino’s classification.
Table 3. Comparison of the PUMCH classification with Tanino’s Type Ib2 is the most common in PUMCH classification.
classification There are 283 cases of type Ib2, which account for 26.23%
PUMCH classification Tanino’s classification
IIa1 Ia and followed by Type IIIa and Type IIIb, of which there
Ib1, Ib2 Ib are 185 cases and 176 cases, accounting for 17.15% and
Ia1 Ic 16.31%, respectively. These three subtypes amount to 644
Ib3 Id cases, which account for 59.69%. In other subtypes, the
IIIa II
proportion of Type Ib1, Type IIa2, and Type IIb exceeds
IIIb III
IVa IVb IV 5%; these types are common. In PUMCH classification,
Ia2, Ic, IIa2, IIa3, IIb – pigmentation macules of nevus of Ota not only involved
3914
the innervation area of the ophthalmic and maxillary nerves
which are trigeminal nerve branches, but also involved the
mandibular nerve which is never mentioned in the world
literature. In Types Ic, IIb, IIIa, and IIIb of the PUMCH
classification, the innervation area of the mandibular nerve
is involved to varying degrees. The new classification
is simple and comprehensive. It is a non-invasive and
feasible method of describing the extent and full view of
nevus of Ota and is convenient for researchers in academic
exchanges.
Both the trigeminal nerve and melanocyte were originated
from the neural crest. The PUMCH classification is based
on the innervation area of the trigeminal nerve branches.
Pigmentation macules occur in the innervations area of
these branches. Therefore, we conjecture that abnormality
that occurred in the melanocyte migrating from the neural
crest might be related to trigeminal nerve cell development.
No relevant reports were found in the literature, and it is
expected to be the focus of future studies of pathogenesis of
nevus of Ota.
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(Received May 7, 2013)
Edited by GUO Li-shao