CASE PRESENTATION

NEVUS PIGMENTOSUS

Clinical Supervisor :
Dr. Herry Setya Yudha Utama, SpB, MHKes, FInaCS

Created by :
Khairul Huda
1102013148

CLINICAL CLERKSHIP OF SURGERY DEPARTEMENT

FACULTY OF MEDICINE YARSI UNIVERSITY
ARJAWINANGUN DISTRIC GENERAL HOSPITAL 2017

1
 CASE PRESENTATION

I. IDENTITY

Date of hospital entry : July, 3rd, 2017

Name : Miss D

Age : 15 years

Gender : Female

Addres : Kebonturi

Religion : Islam

Marital status : Single

II. ANAMNESIS

Main complaint

Patient complain of a big mole in the right back.

History of disease

Patients come to Arjawinangun hospital with complaints of a big mole on right

back. Its make her uncomfortable, so she intend to remove it.

History of past disease

There is no pain, she had no history of surgery and she never had a history of

hypertension and diabetes.

History of family disease

Miss. D said, there is no family members with the same as patient.

2
III. PHYSICAL EXAMINATION

a. Present Status

Genereal condition : No pain

Awareness : Compos mentis

Blood pressure : 110/80 mmHg

Pulse : 80 x/minute, regular

Respiratory : 24 x/minute

Temperature : 36,8 oC

Head

Form : Normocephale, symmetrical

Hair : Black, no hair fall

Eye : Anemic conjungtivas (-/-), icteric schleras (-

/-), light relexes (+/+), isochore pupil right =

left

Ear : Normal form, cerumen (-), thympany

membrane intact

Nose : Normal form, septum deviation (-),

epitaxis(-/-)

Mouth : Normal

Neck

Enlargement of lymph nodes (-), trachea in the middle, no mass found

Thorax

Lungs pulmonary

Inspection : the chest is symmetrical both left and right.

On the right back there is a big mole.

3
Palpation : fremitus vocale and tactile are symmetrical,

crepitation (-), tenderness (-), rebound

tenderness (-)

Percussion : Sonor sound in both lung fields

Auscultation : Vesicular abd bronchial sound in the entire

lung field, ronchi (-/-), wheezing (-/-)

Cor
Inspection: Ictus Cordis not visible
Palpation: Cordis Ictus palpable
Percussion: cardiac boundaries within normal limits
Auscultation: HS I-II Reg G (-) M (-)

Abdomen

Inspection : Flat, symmetrical, mass (-)

Palpation : Tenderness (-), rebound tenderness (-)

Percussion : Tympanity sound in four quadrants

Auscultation : Intestine sound (+)

Extremities

Upper

Muscle Tone : normal

Movement : active / active

Mass :-/-

Strenght :5/5

Oedema :-/-

Lower

Muscle tone : normal

4
 Movement : active / active

Mass :-/-

Strenght :5/5

Oedema :-/-

Genitalia

No abnormalities

b. Localized Status

Regio : infrascapularis dextra

Inspection : Mass appears with 5x7 cm size, black, and

there are no signs of inflammation

Palpation : Palpable masses with flat surfaces

c. Laboratory Examination

Routine Haematology

Test Result Unit

Full Blood
Hemoglobin 13,6 gr/dl
Hematocrit 35,6 %
Leukocyte 7,1 10e3/L
Trombocyte 312 10e3/L
Erythrocyte 4,53 mm3
Erythrocyte Indexes
MCV 83,9 Fl
MCH 27,8 Pg
MCHC 33,2 g/dl
RDW 13,6 Fl
MPV 6,7 Fl
PDW 17,8 Fl
Counts (DIFF)
Eosinophil 2,4 %
Basophil 0,1 %
Segmen 70,8 %
lymphocytes 18,0 %
Monocytes 8,7 %
Stab 0 %
Coagulation

5
 Clotting time 4 menit
Bleeding time 2 menit
Immunology
HBsAg 0,01
Anti HIV non reaktif

X-rays (April 17, 2017)

impression:
There does not appear active pulmonary TB
There does not appear enlarged heart
There does not appear intrapulmonary metastases

IV. DIAGNOSIS

Nevus Pigmentosus

V. TREATMENT

Operative

Excision

Medicamentosa

Ceftazidine, ketorolac and ranitidin.

VI. PROGNOSIS

Ad vitam : ad bonam

Ad sanationam : ad bonam

Ad fungsionam : ad bonam

6
 NEVUS PIGMENTOSUS

A. Background
Skin is the outer organ to protect human body. It had 3 layers; Epidermis, dermis
and hypodermis. Melanocytic nevi are benign neoplasms or hamartomas composed of
melanocytes, the pigment-producing cells that constitutively colonize the epidermis.
Melanocytes are derived from the neural crest and migrate during embryogenesis to
selected ectodermal sites (primarily the skin and the CNS), but also to the eyes and the
ears. Ectopic melanocytes have been identified at autopsy in the gastrointestinal and
genitourinary tracts. Congenital melanocytic nevi are thought to represent an anomaly in
embryogenesis and, as such, could be considered, at least in a sense, malformations or
hamartomas. [1] In contrast, most acquired melanocytic nevi are considered benign
neoplasms. Compare the images below. Melanocytic nevi occur in all mammalian species
and are especially common in humans, dogs, and horses.

B. Definition
Nevus pigmentosus is a benign tumor consisting of melanocytes, pigment-producing cells
that colonize the epidermis.

C. Epidemiology
Frequency

United States
Acquired melanocytic nevi are so common that some authorities believe they cannot be
considered a defect or an abnormality. However, despite the high prevalence, melanocytic
nevi are nonetheless pathologic in the sense that they represent an aberrant or neoplastic
proliferation of cells. Most persons with light skin have at least a few melanocytic nevi.
Melanocytic nevi also occur in dark-skinned individuals, albeit with low prevalence.
Interestingly, there are distribution differences in melanocytic nevi between light-skinned
and dark-skinned individuals. In light-skinned individuals, most melanocytic nevi occur on
the trunk. In dark-skinned individuals, acral melanocytic nevi are relatively more
common. [2]
International

7
The international prevalence of melanocytic nevi is believed to be similar to that observed
in the United States. The prevalence in ethnic groups with dark skin is lower than that
observed in individuals with fair skin. If ultraviolet exposure represents an inducing agent
for the development of melanocytic nevi, than this is unsurprising. Some individuals of
northern European extraction, especially those from northern Germany, Holland, Belgium,
and the United Kingdom, not uncommonly have large nevi ( 1 cm in largest diameter),
often of large number (>50, up to several hundred), with a red-brown color. These nevi
have been called atypical moles or dysplastic nevi.

Race

Melanocytic nevi are common lesions in patients with light or fair skin and are less
common lesions in dark-skinned individuals. This difference in prevalence is in part
attributable to the fact that identifying moles in dark-skinned patients is often difficult,
especially if the lesions are macular (flat).
Some authorities have suggested that melanocytic nevi are in part stimulated by exposure
to sunlight. [3, 4] If so, then individuals with dark skin might have fewer nevi because of the
protective properties of melanin.
Evidence indicates that broad-spectrum sunscreens attenuate the development/evolution of
melanocytic nevi when used in children [5] ; therefore, dark-skinned individuals probably
have inherent protection against the development of moles because of their cutaneous
melanization.

Sex

No clear sex predilection is reported for the development of melanocytic nevi. However,
melanocytes have been postulated to exhibit some degree of sex hormone responsiveness.
The findings associated with melanocytic nevi during pregnancy support this conclusion.
Melanocytic nevi commonly darken and/or enlarge during pregnancy. Melanocytes have
been shown to have cytosolic receptors for estrogens and androgens, and melanogenesis is
responsive to these steroid hormones. Some melanomas seem to respond to hormones, an
observation that might be explained by these cytosolic receptors.

C. Etiology

8
The etiology are unknown cause. Genetic factors are likely in many people, and sunlight
exposure in childhood. Skin naevus cells originate in neural crests, these cells forming
small nests in the epidermal basal cell layer and in the dermo-epidermal dome zone . These
cells divide and enter the dermis and form dens in the dermis. 3
Ultraviolet exposure is thought to be the triggering factor of pigmentosus nevus, seen from
some individuals from northern Europe, especially those from Germany, the Netherlands,
Belgium and the UK, often having large nevus (largest diameter 1 cm), often in large
quantities (> 50, up to several hundred), with red-brown color. This nevus is called an
atypical nevus or a dysplastic nevus.

C. Patofisiolgy

Melanocytes are present in the basal layer of the epidermis and exhibit a certain degree of
territoriality. Non-neoplastic melanocytes typically exhibit contact inhibition to each other,
and thus pigment cells are usually not found as contiguous cells. With certain forms of
stimulation, such as the exogenous administration of ultraviolet irradiation, the density of
melanocytes in normal epithelium may increase. Normal melanocytes may also involve
adnexal epithelium, most notably the bulbs of follicular papillae.
Melanocytic nevi represent proliferations of melanocytes that are in contact with each
other, forming small collections of cells known as nests. Melanocytic nevi commonly form
during early childhood. Their onset is believed by some authorities to be, at least in part, a
response to sun (ultraviolet) exposure. However, genetic factors are also clearly involved
in the development of some types of melanocytic nevi. Some kinships express an
autosomal dominant condition (the so-called dysplastic nevus syndrome or the familial
atypical multiple mole and melanoma syndrome), in which members have a large number
of large nevi, sometimes hundreds, scattered over the integument.
Melanocytic nevi have also been observed to develop or spread rapidly after blistering
events, such as second-degree thermal burns, severe sunburns, or toxic epidermal
necrolysis or in persons with genetic blistering diseases such as epidermolysis bullosa. In
such instances, the development of so-called eruptive melanocytic nevi appears to be
propagated by a traumatic stimulus, with scattering of melanocytic nevus cells over a large
area within a zone of blistering and with the subsequent development of multiple
independent melanocytic nevi within the injured area. Growth factors, such as basic

9
fibroblast growth factor, have been suggested to be released by proliferating keratinocytes
and could contribute to stimulation of melanocyte proliferation in this context. In
summary, the exact etiology behind the development of melanocytic nevi is complex and
multifactorial and is incompletely understood.
Acquired melanocytic nevi are considered benign neoplasms. In contrast, congenital
melanocytic nevi are perhaps best interpreted as congenital malformations. Melanocytes
are of neural crest origin, and congenital nevi probably represent an error in the
development and migration of these neuroectodermal elements. Evidence of errant
embryological migration can be seen histopathologically within giant congenital
melanocytic nevi. In this context, melanocytes may be found distributed throughout the
dermis, around and within the walls of blood vessels, around adnexal structures such as
hair follicles, within the subcutis, and sometimes within skeletal muscle, smooth muscle
bundles, nerves, or sebaceous glands.
Errant embryological migration is also believed to be the source of melanocytic nevus cell
"rests," which can be observed in the capsules of lymph nodes. Occasionally, rests of
melanocytes can also be found in the subcapsular space or within lymph node trabecula.
The importance of melanocytic nevus rests is that they can sometimes be mistaken for
metastatic deposits because of their extracutaneous location.
These rests are not uncommonly associated with agminated blue or cellular blue nevi or
with large congenital melanocytic nevi. However, with the advent of sentinel node
evaluation, nodal rests of melanocytic nevi clearly are not uncommon and can be found in
association with a variety of melanocytic and nonmelanocytic lesions. These rests of cells
are sometimes referred to as benign metastases because these cell clusters may represent
the end result of an intralymphatic migration of benign melanocytes. Note, however, that
benign nodal melanocytic nevi are almost invariably within the capsule, while melanoma
metastases are commonly subcapsular.

Melanocytes are in the basal layer of the epidermis. Non-neoplastic melanocytes typically
show inhibition of contact with each other, and thus melanocyte cells are not usually found
adjacent. With certain forms of stimulation, such as ultraviolet radiation, the density of
melanocytes in the normal epithelium may increase. Normal melanocytes may also involve
the adnexal epithelium, particularly the tip of the follicular papilla.1

10
Nevus pigmentosus is a proliferation of adjacent melanocytes, forming a small collection
of cells known as nests. Nevus pigmentosus usually forms at an early age. One of the
trigger factors is believed to be exposure to sunlight (ultraviolet). However, genetic factors
are also clearly involved in the development of several types of pigmentosus nevus. 3
Several generations (descendants) express an autosomal dominant condition (so-called
dysplastic naevus syndrome or familial atypical multiple mole and melanoma syndrome),
in which family members have large, sometimes hundreds, of nevus scattered in the skin. 1
Acquired acquired pigmentosus (acquired melanocytic nevi) is considered benign
neoplasm. Conversely, congenital pigmentosus nevus may be interpreted as a congenital
defect. Melanocytes are derived from neural crest, and congenital nevus may be a form of
error in the development and migration of neuroectodermal elements. Evidence of
embryological migratory errors can be seen histopathologically in giant congenital
pigmentosus. In this case, melanocytes can be distributed throughout the dermis, around
and inside the blood vessel wall, around the adnexal structures such as hair follicles, in
subcutis, and occasionally in striated, smooth muscle, nerve, or sebaceous glands. 1

D. Clinical manifestations

Nevus pigmentosus can occur in all parts of the skin of the body, including the mucous
membrane near the surface of the body. Lesions may be flat, papular. Or papillomatous, is
usually 24 mm in size, but may vary from the pin to the size of the palm. Pigmentation
also varies from skin tone to dark brown. 3 The born naevus is defined as a congenital
nigus pigmentosus. Some scientists also include nevus pigmentosus in the congenital
category if the nevus arises before 6 months after birth.5 According to size it can be
divided into 2 groups: small lesions when the nevus diameter is smaller than 1.5 cm to 20
cm, and large lesions (giants) When the diameter is more than 20 cm.

11
The acquired pigmentosus nevus is usually less than 1 cm (often <6mm) in size, with a
smooth, regular surface. Depending on size and elevation, the acquired pigmentosus nevus
may be seen as a macula, papule or nodule. 5 Histologically the pigmentosus nevus may be
divided into intradermal nevus; Nevus junctional; And mixed nevus. Clinically these forms
are difficult to distinguish. The junctional nucus is usually dark brown in the shape of a
macula. The slightly prominent lesions, the brown color, and the shaped papules or
nodules are mixed nevus, whereas intradermal nevus almost always has a stalk almost and
has no specific color.6,7
Nevus junctional
Generally hairless, macular to dark brown, the size varies from 1 mm to 1 cm (diameter),
smooth and flat surface. Lesions are usually round, elliptical, small, irregular. The location
is often in the palms, soles of the feet and genitalia. Junctional nucus is rare after birth and
usually develops after 2 years of age.

(Nevus junctional)

Mixed Nevus (Compound) Almost the same as the junctional nevus but slightly prominent
and some are papilomatous. The color is like the color of the skin until brown. Smooth
surface, many locations on the face and usually overgrown hair. Cell nevusnya barada on
epidermis and dermis.

12
Nevus intradermal
The papul shape (dome), size varies from several mm to 1 cm or more (diameter).
Locations where-man but at most in head, neck and usually overgrown with coarse, dark
brown hair. The nevus cell is in the dermis.

Basically, pigmentous nevus does not provide symptoms if the nevus is benign, unless the
pigmentous nevus is malignant it will arise symptoms such as; Scalp, mucosal,
anogenital lesions; Growing fast; Color varies; irregular edge; Erosion in lesions
without major trauma; persistent itching, pain, bleeding; Dermoscopy: melanoma or
dysplastic. 3 Nevus pigmentosus obtained following the ABCD nevus rule (A symmetry,
B order irregularity, C olor variation, size / D iameter, and E valanceary change). The
existence of deviations from these instructions should be given attention. In this case a
biopsy is necessary for histopathological examination

13
E. Treatment

Management of nevus is usually associated with cosmetic terms, or the possibility of a

nevus turning into a malignancy. Most melanocytic lesions do not require special therapy.
Appointment of nevus through excisional or excision biosynthesis techniques of electro
dessication excision or complete elliptical exudation (depending on size, tendon and lesion
location).

F. Prognosis

Generally good. But in the junctional nevus and nevus compound should get attention
because there is likely to turn into malignant.

14
 DAFTAR PUSTAKA

1. Utama, H., S., Y., 2010. Skin Disorders [cites 19 July 2017] [Available from :
https://herrysetyayudha.wordpress.com/tag/skin-disorders/ ].

2. McCalmot, Timothy. 2013. Melanocytic Nevi. Journal of Cutaneos Pathology. San

Fransisco.

3. Siregar, RS. 2013. Atlas Berwarna Saripati Penyakit Kulit, E/2. Jakarta: EGC.

4. Wolff K, Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ. 2008.

Fitzpatricks Dermatology in General Medicine. 7th Edition. New York: McGraw Hill.

p 1054-67.

5. Weller R, Hunter J, Savin J, Dahl M. 2008. Clinical Dermatology . 4th Edition.

Australia: p297-294.

6. Viana ACL, Gontijo B, Bittencourt F. Giant congenital melanocytic nevus. AN Bras

Dermatol. 2013; 88 (6):863-78

7. Leleux TM. Pathology of Benign Melanocytic Nevi: overview, pathophysiology, and

etiology, epidemiology. Medscape Reference. 2013.

8. Wolff K, Johnson RA, Suurmond D. 2007. Fitzpatricks Dermatology Atlas. McGraw-

Hills.

9. Curry JL. Pathology of Dysplastic (Atypical) Melanocytic Nevi. http://emedicine.

medscape.com/article/1960604-overview. 2013 Oct 30.

10. Gerardo F, Argenziano G. 2007. Blue Nevus. ColorAtlas of Melanocytic Lesions of the

Skin. New York: Springer.

11. Fung MA. Pathology of spitz nevi: overview, pathophysiology and etiology,

epidemiology. http://emedicine.medscape.com/article/1963323-overview. 2013 Jun 06.

15