Prof.

Ossama
Hussein Roshdy
 It is a term given to a wide range of
disorders all are characterized by
cutaneous hypopigmentation.
Melanin is produced from
Tyrosine.
Density of Melanocytes in the skin is the
same for all human races.

Melanocytes originate from melanoblasts.

Melanocytes in the skin, oral mucosa, and

uvea derive from spinal portion of the
neural crest.
Tyrosine DOPA DOPA quinone

DOPAchrome

DHI + DHICA Melanin.

 INFANCY ( Diffuse-CIRCUMSCRIBED).

 CHILDHOOD (Diffuse/ CIRCUMSCRIBED).

 ADULTHOOD (Diffuse/ CIRCUMSCRIBED).

 INFANCY / Diffuse

Eyes, skin & Hair Skin &/or Hair

Menkes Griscelli ‘s – Nutritional Sialic a

Elejalde Deficiencies,
syndromes Storage
e.g. Se, Disease
Copper
EEC
OCA
Phenylketonurea
(PKU)

1 2 3 Hermansky Pudlak Cross

Chediak - Higashi
Prader – Willi
Angelman
 INFANCY / CIRCUMSCRIBED

Lack of Pigment Decreased

Pigment

Vitiligo Piebaldism Post Post-

Infectious Inflammatory

Ash leaf Hypomelanosis

Nevus
spots Of Ito
Depigmentosus

Waardenburg Waardenburg
+
Hirshsprung
 CHILDHOOD / CIRCUMSCRIBED

Diffuse Circumscribed

Total Body Post- Post- Post

Vitiligo Infectious
Vitiligo Inflammatory Traumatic

Scleroderma

VKH Chemical Melanoma-

Leukoderma Associated
leukoderma
 ADULTHOOD

Vitiligo Post- Post- Post

Traumatic Inflammatory Infectious

Atopic Lichen
dermatitis Psoriasis Eczema PLC
striatus
LS & A
 Oculocutaneous
Albinism

- White hairs.
- Milky white skin.
- Blue-grey eyes.
Chediak – Higashi Syndrome

- Rare syndrome.
- Inherited as an autosomal recessive.
- Bleeding tendency.
- Platelet dysfunction.
- Progressive neurologic dysfunction.
- Severe immunodeficiency.
- Marked susceptibility to respiratory and
cutaneous infections.
- Uusually fatal before the age of 10 years.
- later on  from a malignant lymphoma.
 Piebaldism

- Autosomal dominant.
- Mutatio n on
chromosome 4q11-12.
- Since birth.
- Mainly forehead,
trunk, and extrimities.
- Triangular or diamond
shaped areas.
- Usually on front
of the body.

- Specially on
midline
distribution.
- Whit forelock.
- May be  poliosis of eyebrows and
eyelashes.
- Treated mainly surgically.
 Waardenburg Syndrome

- Rare, autosomal
dominant or A.
recessive.

- Achromia of hair and/or

skin.

- Broad nasal root 

dystopia canthorum.
- Heterochromia irides.
- Congenital
deafness.

- Medial eyebrows
hyperplasia.
 Post Infectious lekoderma

- Superficial fungal infection.

- Pityriasis versicolor.

fung
us
- Unsaturated fatty acids
oxidation Azelaic
acid

products  inhibit tyrosinase activity.

 Others:
- Leprosy.
- Pinta.
- Yaws.
- Bejel.
- Secondary stage Syphilis.
 Naevus
depigmentosus

- It is a misnomer.
- Usually present at birth.
- Single but may be multiple,
circumscribed.
- Rounded, dermatomal or in whorls
and streaks.
Ash leaf spots (tuberous
sclerosis)

- It is an autosomal dominant disease.

- Multiple, irregularly
scattered(ashleaf appearance).
 Vogt-Koyanagi syndrome
(HARADA)

- A disease of unknown etiology.

- It affects 4 organs in succession:

1- Meninges encephalitic
or meningitic symptoms +
lymphocytosis of CSF.
2- Eyes
bilateral uveitis,
choroiditis and
optic neuritis(some
recovery of visual
acuity).
3- Inner ears
deafness and/or tinnitus (over 50%
of cases) completely restored.
4- Skin (permanent
changes)

vitiligo (60% of
cases), poliosis (80%
of cases) and
alopecia areata (in
50% of cases).
 Positive family member affection in
about 25-33% of cases.
 No prove of autosomal (recessive-
dominant) trait.
 Multifactorial inheretance genetic
pattern is postulated (on
chromosomes 1-2-and 4).
Heterogeno
us-
Convergenc
e theory
I- Auto-immune
theory:
- Association with some auto-immune diseases.

- Antibody activity is more pronounced in active

rather than stable disease.
- ↑ Antibodies against melanocyte
surface antigens.

- ↑ Antibodies against common tissue

antigens (thyroid, gastric parietal
cells and adrenal tissue) in 80% of
vitiligo cases.

- ↑ Level of anti-tyrosinase antibodies.

- T-cell profiles are abnormal in vitiligo
with a decrease in T-helper cells.

II- Auto-cytotoxicity
theory:

- Melanocyte activity
Melanocyte death.
 Melanocyte
death
↑ • Inhibition of
Melanocyte Thioredoxin
reductase enzyme (a
susceptibili free-radical
ty to the scavenger)
precursor ↑accumulation of free
molecule radicals.
(Dopachro
me)  cell • ↓Level of catalase
death. (which reduces
superoxides).
↑ Dopamine auto-
oxidation.
III- Neural
theory:

• Depigmentation in animal models

with injured nerve fibers.
• Nerve injury associated absence of
vitiligo lesions.
• Segmental dermatomal type of
vitiligo.

• Increased adrenergic activity

(sweating – vasoconstriction) in vitiligo
areas.
 Evidenced by:

Elevated levels of in Melanocytes

of Neuroten
sin TNF-α
vitiligo lesions may suggest neurogenic control.
 Vitiligo areas

Absent Melanocytes.
Vitiligo skin Normal skin
 Vitiligo. A melanocyte with a giant
melanosome is present at the edge of the
depigmented area. (H&E)
• Cultured Melanocytes at lesion’s
peripheries
Poor growth
+ premature
death

Evidenc
Degeneration:
e of vacuolization
Pyknosis
melanosomes
aggregation
 Sometimes  ?? Inflammatory
changes at the borders of lesions.
 Onset  childhood or young adults.
 in ≈20% develops after severe
sunburn or severe emotional or
physical stress.

 Incidence  ↓ with increasing age.

 Gender  preponderance in
female patients (?? False
preponderance).
 Prevalence is variable from area to
another:
 USA → 1%.
 Denmark →
0.38%.
 India →
1.13%
 According to the extent of involvement:

1- Generalized.
2- Universal.
3- Acrofacial (distal fingers + facial orifices).
4- Segmental (dermatomal-asymmetric).
5- Focal (localized non-dermatomal).
6- Vitiligo gradata (trichrome
type).
7- Vitiligo with raised
borders.
8- Drug induced vitiligo – rare type
(Chloroquine- Clofazimine).

9- Chemical- induced leukoderma

(occupational):

- Phenolic compounds (p-TBP)-

monomethyl ether of hydroquinone)

- Sulfhydryls (Sulfanilic acid –

Mercaptoethylamine MEA).

- Others: arsenic- corticosteroids – azelaic

acid – mercurials.
10- Vitiligo with melanoma:

-Regression or slow progression of

melanoma
long survival time .
-Halo nevus.
-Diffuse depigmentation and/or hypo
pigmentation (remote from the
tumor)
 CLINICAL SYSTEMIC
ASSOCIATIONS

A- Autoimmune diseasaes:
- Addison’s disease.

- Thyroid disease.

- Diabetes.

- Alopecia areata.

B- Uveitis.
C- Auditory problems.
D- Vogt-Koyanagi-Harada Syndrome.
 Ophthalmologic
examination


Wood’s light
ENALUATION

examinatiuon
Complete blood
picture

Psychological
evaluation

TSH level
 TREATMENT

Phototherapy
Cosmetic
•Psoralen UVA
camouflag
•Narrowband
e
UVB

Topical and
Surgical
systemic
treatment
steroids
I- Cosmetic
camouflage

Cover mark-
Type of Dermablend –
dyes Dermacolor -
Dermage

On exposed areas
Sites of (face, neck, and
application hands)

Personal rejection
Drawbac Severe reaction
ks Not practical for
extensive lesions
 PUVA
therapy Systemi
c
P = Psoralens
Topical

UVA = Ultraviolet
A 360 nm
waveleng
th
 8 methoxy-psoralen 0.5 mg/kg.
 Used for extensive vitiligo.

Exposure:
• 1.5-2 hours after
ingestion of the drug.
• 1-2 J/cm2
increments by 0.25 J/cm2 till
erythema dose.
• 2-3 times / week.
 8 methoxy-psoralen 0.05- 0.1%
solution.
 Used in cases with less than 20%
total surface area depigmentation.
Exposure:
• Duration before exposure is
not important.
• 1-2 J/cm2 .
• 30 seconds
increments by 15-30 seconds up
to 10 minutes.
• 2-3 times /week.
 PUVA increase the size but not the
number of Melanosomes.
1-
photoadducts
UV light
 Psoralen + DNA
photoadducts

(with thymine bases) DNA

inhibition

RNA + protein
synthesis.
2-
immunologic :
Cytokines release from keratinocytes:
 Decreasing the antibodies directed
• Interleukin-1 Facilitates
binding of Melanocytes.
against
α-MSH to
keratinocytes.
• Leukotriens C4, D4 Stimulate
Melanocyte

proliferation (in-vitro).
• Endothelin-1 Proliferation
and

differentiation of

Melanocytes.
 SIDE
EFFECTS

IMMEDIATE DELAYED

•Erythema.
•Pigmentati • Skin
on. aging.
• Fatigue. •Malignan
• Xerosis. cy (SCC).
• Pruritus. • Cataract
PUVASOL

Exposure:
- We use 5 MOP or TMP.
- Dose of 0.3 mg/kg.
- 2-4 hours outdoor light
exposure (10 AM – 3 PM).
- 5-10 minutes
increments by 5 minutes/ session for 4
exposures gradual
increase up to 2 hours.
Narrowband UVB (NB-
UVB)
 Usually 311-312 nm wavelength.
 Treatment of choice for adults and children with
generalized vitiligo.
 250 mJ/ cm2 increments by 15%
at each exposure erythema.
 2-3 times /week.

1-Advantag
Can be used in children.
2-es
Can be used in lactating and pregnant women.
3- Can be used in hepatic or kidney dysfunction.
 Repigmentation
occurs from hair
follicles.

Upward migration of
Melanocytes is influenced
by cytokine release from
keratinocytes.
 Topical and systemic
steroids
 For localized lesions.
 Face and neck may respond better.
 High potency topical corticosteroid
preparations (0.1% betamethasone
valerate -0.05% clobetasol
propionate) are effective.
 1-2 months  tapering.
 IM corticotropins may also help.
 Surgical
treatment
INDICATIONS

 Segmental or localized vitiligo.

 Non progressive-inctive disease.
 In areas such as:
- Dorsal aspect of fingers.
- Forehead.
- On hair lines.
- Ankles.
Surgical treatment
modalities

A- Epidermal
Grafting
B- Autologus
Minigrafting
D- Transplantation of cultured
Melanocytes

C- Transplantation of non-cultured
Melanocytes