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Pigmentation Disorders

BY
Dr.Ahmed Noureldin Ahmed
MBBS, DCH ,DTM&H
UMM-GHOILINA H.C
.
 Melanocyte
.It is the melanin – producing cell►
.It lies in between basal Keratinocytes ►
It is a dendritic cell►
The dendrites transfer Melanin to Keratinocytes, to►
.protect their nuclei from the destructive effect of UVR
The colour of the skin depends on the►
,rate of melanin production & distribution
but not the number of melanocytes
 Melanin
is a brown-black, light-absorbing pigment,
protecting the skin against ultraviolet rays
The melanin system is composed of functional
.units called epidermal melanin units
Each unit consists of a melanocyte that
supplies melanin pigment to a group of
.keratinocytes
Pigmentation is determined primarily by the
amount of melanin transferred to the
keratinocytes
 Pigmentary Disorders
A- Hypopigmentation
Vitiligo- 1
Post-inflammatory Hypopigmentation- 2
Oculocutaneous Albinism- 3
Idiopathic Guttate Hypomelanosis-4
Hypopigmented Macules in Leprosy- 5
 A- Hypopigmentation
1- Vitiligo
An idiopathic, circumscribed hypomelanosis of
skin and hair.
It may be an autoimmune disease and is
associated with other autoimmune diseases:
pernicious anemia, DM, and Addison’s dis
Peak incidence is 10 to 30 years of age.
Occurs in all races but is most cosmetically
disfiguring in darker-skinned people.
 Vitiligo
An idiopathic inflammatory
Process that results in
skin depigmentation
consequent to loss of
melanocytes from
the epidermis
and in whitening of hair that follows loss of
. melanocytes from the bulb of follicles
 Vitiligo
It presents with milky-white patches of►
varying sizes and shapes on any
.body site
There is no history of preceding►
.inflammation
Lesions are often symmetrical and►
frequently involve the face, hands and
.genitalia
 .(Vitiligo ( cont
The hair can also depigment►
Trauma may induce new lesions.►
►Spontaneous repigmentation can occur
and often starts around hair follicles giving
.a speckled appearance
Repigmentation is rare if a lesion has►
persisted for more than 1 year or if the hair
.is depigmented
(Vitiligo (cont
.
 Treatment
It was an Egyptian, Professor Abdel Monem
El Mofty, Department of Dermatology,
Cairo University Medical School, who
observed plants used in Egyptian folk
medicine and began the development of
modern photochemotherapy (PUVA( for
vitiligo and psoriasis. In the 1940s, he
used crystalline methoxsalen
(Meladinine( followed by sunlight
exposure to treat vitiligo
 Treatment
.spontaneous repigmentation may occur- 1
Topical Psoralens and topical Steroids- 2
Photochemotherapy ( PUVA( or Psoralens- 3
.and exposure to natural sunlight
:Surgical Treatment- 4
a( Minigrafting
b( Cultured Melanocytes
(c( Permanent micropigmentation (Tattoing
 Treatment
► Narrow-band UVB for repigmentation
of generalised vitiligo
►new immunomodulatory agents, such
as calcineurin antagonists, have
recently been introduced as new
promising tools to treat acquired
hypopigmentary disorders
 Post-inflammatory- 2
Hypopigmentation
One of the most common
. causes of pale skin
It is much
common in people
with pigmented skin
It may be seen as
, a consequence of eczema
acne or psoriasis
Providing the skin disease is controlled , the
pigmentation will recover slowly after many months
 Post-inflammatory
hypopigmentation
.
 Oculocutaneous Albinism- 3
autosomal recessive disorders affecting the
. pigmentation of skin, hair and eyes
It can affect all races☻
Melanocytes are in normal number but have♣
. abnormal function
Clinically it presents with universal pale♠
. skin, pale blonde hair and a pinkish iris
Photophobia, nystagmus and a squint are♥
. also present in most cases
 Albinism
.
 Treatment
involves obsessive protection against

Sunlight to avoid sunburning and

development of skin cancer

Asymptomatic
Multiple
scattered
Hypopgmented
macules on the legs
Due to idiopathic
hypomelanosis
 Leprosy- 5
Both tuberculoid leprosy and indeterminate
leprosy can present with anaesthetic patches
.of depigmentation
Loss of hair and decreased sweating may ►
also be present in the lesions
Skin lesions are numerous, varying in size and►
( form (macules, papules, plaques
annular, rimmed lesion with punched-out,►
hypopigmented anaesthetic centre is
characteristic
 Leprosy
Leprosy (Hansen's disease(
is caused by the
acid-fast bacillus
Mycobacterium leprae.
Multiple asymmetrical
hypopigmented
Anaesthetic patches
Are seen in
Tuberculoid type
 -Pityriasis Versicolor 6
well demarcated symmetric hypopigmented
minimally scaly papules and plaques
 Pityriasis Alba- 7
is a nonspecific dermatitis of unknown etiology that
causes erythematous scaly patches.
These resolve and leave areas of hypopigmentation .
It is more prominent in dark-skinned patients
It occurs predominantly in children 3-16 yrs
Most cases persist for several months
Several patches are usually observed and confined to the
face , around the mouth and cheek, but can be seen on
the trunk and limbs
Pityriasis Alba
.
 Treatment
►Pityriasis alba resolves spontaneously and
may not require treatment .
►Apply Simple Emollient Cream(Aquaderm(
2-6 times/day( to retain moisture in the skin(
►A mild Tar Paste may be helpful for chronic
cases on the trunk.
►Hydrocortisone 1% Cream , bid for a wk may
be helpful .
►Photoprotection may be considered
 LEUCOGO Cream
►Natural skin care cream for
depigmentation,
to help in the regulation of
Depigmentation
► Sufficient amount to be applied
once daily at sun set ( layer on
layer (
 B- Hyperpigmentation
1- Freckles
2- Lentigos ( Solar Lentigo – Lentigo Maligna(
3-Chloasma
4- Postinflammatory Hyperpigmentation
5- Metabolic and endocrine effects
6- Peutz – Jegher Disease
7-Urticaria Pigmentosa
8- Acanthosis Nigricans
9- Mongolian Spots
 1- Freckles(Ephelides(
These appear in childhood as small
brown macules after sun exposure.
They fade in the winter months.
 -Lentigos 2
These are a more permanent macule of
pigmentation similar to freckles but
they tend to persist in the winter
 Solar Lentigo
Flat hyperpigmented
macules occur in
older people on
sun exposed areas
Because of actinic
damage
 Lentigo Maligna
Flat asymmetrical
dark brown macules
On the face
ٍBiopsy of the lesion
Revealed Lentigo
Maligna
 Treatment of Lentigos
►Liquid Nitrogen (Melanocytes freeze at -4ºC
►Krypton Laser
► Tretinoin cream( Retin A( and hydroquinone
cream can lighten lentigines. Retin A is a
keratolytic agent Acts by increasing epidermal cell
mitosis and turnover while suppressing keratin
synthesis. Important side effect is hypopigmentation,
which reduces the appearance of lentigines.
►ٍSunscreen before exposure to sun
►Whitening cream ( Avalon(
contains Hydroquinone 2% , Vit.E and sunscreen
►Unitone Cream with or without sunscreen
 Chloasma- 3
These are brown symmetrical macules
over the cheeks and forehead,
and are most common
in women.
They can occur
spontaneously
but are also associated
with pregnancy
and the oral CP.
 Postiflammatory- 4
hyperpigmentation
Excessive scratching
Of the vesicles of
Chicken pox
Leads to areas of
Hyperpigmentation.
 Metabolic and Endocrine- 5
Effects
1- Haemochromatosis
2-Cushing Syndrome
3- Addison’s Disease
4-Nelson’s Syndrome
 Peutz-Jegher Syndrome- 6
This is an autosomal dominant genetic
condition.
It presents with brown macules of the
lips and perioral region
It is associated with gastrointestinal
polyposis which almost never becomes
malignan
 P-J Syndrome
((cutaneous sign of systemic dis
multiple lentigines
on her lips .
2-4 mm
hyperpigmented
macules
 Urticaria Pigmentosa- 7
((Cutaneous Mastocytosis
This presents most commonly with multiple
pigmented macules in children.
These lesions tend to become red, itchy and
urticated if they are rubbed (Darier's sign(
Occasionally lesions may blister and in the rare
congenital, diffuse form of the disease the
skin may become thickened and leather
Skin biopsy shows an excess of mast cells in
the skin
 Urticaria Pigmentosa
systemic symptoms
are present such as
wheeze, flushing,
syncope or diarrhoea
► The condition
spontaneously
resolves after some
years in children but
is persistent in adults.
Urticaria Pigmentosa
.
 (Acanthosis Nigricans (A.N- 8
AN is characterized by symmetrical,
hyperpigmented, velvety plaques that may occur
in almost any location but most commonly appear
on the intertriginous areas of the axilla, groin and
posterior neck
The posterior neck is the most commonly affected
site in children
The vulva is the most commonly affected site in
females who are hyperandrogenic and obese
A.N
.
 Treatment of A.N
The goal of therapy is to correct the underlying
disease process. Correction of hyperinsulinemia
often reduces the burden of hyperkeratotic
lesions. Likewise, weight reduction in obesity-
associated AN may result in resolution of the
dermatosis.
►Topical Retinoids :
promote shedding of hyperkeratotic skin. They are
modifiers of keratinocyte adhesion, differentiation, and
proliferation
 (Treament of A.N ( Cont
► Topical Dx, → keratolytics (topical tretinoin(.
► Oral agents include etretinate and dietary
fish oils. Dermabrasion and external radiation
also may be used to reduce the hyperkeratosis
►20% Urea cream
►Alpha Hydroxyacid
►Salicylic acid
 Mongolian Spots- 9
An asymptomatic
bluish discoloration
overlying
the sacrococcygeal
area is present
at birth.
 Principals of Topical Therapy
Topical dermatologic treatments are
used as cleansing agents, absorbents, anti-
infective agents, anti-inflammatory agents,
astringents (drying agents that precipitate
protein(, emollients (skin softeners(,
and keratolytics (agents that soften, loosen,
and facilitate exfoliation of the squamous cell(
 Principals of Topical therapy
Topical Therapy consists of an active ingredient, an
appropriate vehicle or base to deliver this, and often a
preservative to maintain its shelf-life
Creams :
a semisolid mixture of oil and water held together by an
emulsifying agent.
A preservative such as parabens is added
They are 'lighter' and rub in more easily than oint.
They are useful for face and hand lesions
►They are less effective than ointments.
 Ointment
►asemisolid , contain no water , based on oil or
greases.
►They are the best treatment for dry flaky skin
disorders
Lotions
►These are based on a liquid vehicle such as water
or alcohol.
►They are usually volatile giving a cooling effect
which is a useful antipruritic
►They are useful for weeping skin conditions and
on hairy skin ( e.g. the scalp (
 Gels
Semisolid preparations of high M/W polymers.
►They are non- greasy and liquify on contact
with the skin
►They are useful for treating hairy skin (e.g. the
scalp (
Pastes
►Contain a high percentage of powder in an
ointment base.
►They are thick and difficult to remove from the
skin. ( e.g. Lassar’s paste( used on plaques of
psoriasis.
 Emollients
are composed of water in oil emulsions
► Emollients function in smoothing the rough
skin, changing the skin's appearance,
lubricating, replacing natural skin lipids,
and providing occlusion.
►They fill the spaces between the
corneocytes, thus providing improvement to
defects in desquamation
 Aerosol
forms of betamethasone dipropionate and
triamcinolone acetonide are available but are
seldom used because they offer no advantage
over creams,lotion and solutions
Solution
are homogenous mixtures of two or more
substances.
 (Hydroquinone ( HQ
► is one of the most widely prescribed skin-
lightening agents in the world .
►It is the most effective inhibitor of
melanogenesis.
►tretinoin is used to enhance the efficacy of HQ
►Other depigmented agents are kojic , azelaic acid,
mulberry , Melatoonin and glabridin
( which is the main ingredient of Licorice extract ,
inhibit tyrosinase activity of melanocytes(
 Guidelines for Topical Steroids
• The face should be treated with mild steroid
• Potent Steroids should be used for short courses.
• You can use very potent steroid on the palm and
soles , as the skin is much thicker.
• Regular use of Emollients may lessen the need for
steroid use .
• Only use steroid on inflamed skin.
• Use mild steroids in flexures
.
.