PIGMENTATION DISORDERS

CUTANEOUS HYPOPIGMENTATION

Onset –

Birth/Infancy

Childhood

Adult

Pattern-

Circumscribed

Diffuse

Cutaneous hypopigmentation at neonate or infancy

<1 year

Diffuse Circumscribed

Skin,hair and eyes Skin and/or hair (-) Pigment ↓ Pigment

OCA PKU Menke’s Vitiligo Post-infectious

1- Histidinuria Griscelli Piebaldism Post-inflamm

2-Prader-Willi Homocystinuria PseudoTLP Waardenburg Nevus depigment-

3-Angelman Nut’def (Se,Cu) osus

4.Chediak-Higashi Ecto.dysplasia WS+Hirsch- Ashleaf macules

5.Cross Sialic acid storage sprung Hypomelanosis Ito/

mosaicism
Clinical phenotypes of OCA-

IA OCA (previously tyrosinase negative OCA)

IB

Hermansky-Pudlak (HPS)

Chediak-Higashi (CHS)

Chediak-Higashi syndrome-

Large intracytoplasmic granules within circulating PMN cells.

Prader-Villi-

MR,paroxysms of laughter

Angelman-

Hyperphagia

CIRCUMSCRIBED HYPOPIGMENTATION, CHILD,1-12 yrs

1.Vitiligo

2.Post-traumatic

3.Post-inflammatory –atopic dermatitis,psoriasis,lichen striatus,LSA,Pityraisis lichenoides chronica

4.Post-infectious

ADULT,HYPOPIGMENTATION

I Difuse-

Total body vitiligo

II Circumscribed-

Vitiligo-scleroderma,Vogt-Koyonogi-Harada syndrome

Leucoderma-chemical induced (adhesives,antioxidants in ruber),melanoma associated

Post-inflammatory

Post-traumatic
 Post-infectious

All diffuse hypopigmentation at infancy

-Normal number of melanocytes

But

Decreased mealnogenesis or decrease in the transfer of melanosomes to keratinocytes

Cause:-

Quantitiative-

Lack of tyrosinase activity (OCA I A) IA =Absence

Qualitative-

Decrease in tyrosinase activity (OCA 1 B)

Or

Tyrosinase dysfunction due to relative deficiency of copper

Decrease in transfer of melanised melanosomes

Pigment dilution-

CHS

Griscelli syndrome (silvercolored hair with multiple clumps of melanin)

Circumscribed hypopigmentation,infancy

Vitiligo,piebaldism-

Absence of epidermal melanocytes (complete lack of pigmentation)

Piebaldism:-

pie=magpie (corvine bird)

Resembling a crow

AD disorder

Congenital leucoderma

-Present at birth

-Remains stable
-Classic distribution pattern viz., midforehead,ventral trunk,midportions of the extremities

Routinely spares the midline of the back

Lesions:

Patches of (islands of ) leucoderma within which macules of normal and hyperpigmented skin (+)

Associations:-

1.WS

2.Hirschsprung disease (Congential megacolon)

Basis-

Neural crest cells –

Melanocytes-Skin,hair follicles,inner ear,irides

Ganglion cells-Large intestine

Piebaldism and/or white forelock-

Look for signs of Waardenburg syndrome(WS) deafness,heterochromia itides,facial dysmorphism

WS I = Piebaldism +heterochromia irides+deafness+dystopia canthorum

WS II = Above all but no dystopia canthorum (II =Two eyes)

WS III=Above all +congenital anomalies of upper limb

Leucoderma of scleroderma:-

Repigmenting vitiligo

Retaining>Repigmenting

Because of its resemblance to vitiligo that has begun to repigment via perifollicular macules
(perifollicular retaining)

Dyschromatosis:-

Both hyperpigmented and hypopigmented lesions are observed together

e.g.,

Pinta,Dyschromatosis universalis hereditaria,Dyschromatosis symmetrica hereditaria

Hirschsprung disease:-
(Congenital megacolon)

Associated piebaldism or WS

Melanocytes Skin,hair follicles,nner ear,irides

Neural crest cells

Ganglion cells Large intestine

Nevus depigmentosus> ash leaf macule-

Single HP macule

Otherwise asymptomatic child

Tuberous sclerosis-

Multiple HP macules

Patches of poliosis

Lance-ovate shape

Confetti macules

Facial angiofibromas

Ungual fibromas

Genes involved-Tuberin,Hamartin

Pathogenesis

Decrease in epidermal melanin content

Due to decrease in the transfer Due to decrease in the number of melanosomes

Nevus depigmentosus Tuberosclerosis

Hypopigmented macules of recent onset with limited or unusual distribution

1.Vitiligo-Koebnerised

2.Halo nevi

3.Poliosis

Greek. Helios=Grey

“Circumscribed area of white hair”

Scalp-Usually in the midline of the frontal scalp (white forelock)

Eyebrows

Eyelashes

Migratory poilosis = forme fruste of alopecia areata

4.AA

Post-inflammatory hypopigmentation in children

1.P.alba

2.Atopic dermatitis in fossae

3.Post-topical CS

4.Lichen striatus

5.Pityriasis lichenoides chronica

6.LSA

7.Pityriasis versicolor

8.HD (PB)

9.IGH

Vogt-Koyanagi-Harada syndrome:-

Cutnaeous depigmentation+poliosis

IIIstage of the disease

H/O Otic disturbance

Inflammation of the anterior or posterior segments of the eye or meninges

Three organs-meninges,ears,skin
Three phases:

I Meningo-encephalitic phase-

Aseptic meningitis-fever,headache,malaise,nausea,vomiting,

II Bilateral uveitis-

Choroiditis,optic neuritis,disacousia

III Phase-

Poliosis,leucoderma,alopecia

[At Green Park]

Melasma-

Transient melasma and Persistent melasma

Paracrine influences

Adjacent cells- Keratinocytes+Fibroblasts+Melanocytes

Frictional dermatosis

?Journal reference

Freckles vs lentigines

Lip pigmentation-

Familial

Acquired

Tinosorb x UVA +VL

Oral isoretinoid DOES reduce facial pigmentation

Mequinal-

Derivative of HQ

Can be combined with RA

Straight to procedures indications-

(i)Pigment demarcation lines

 (ii)Pseudoochronosis

(iii)Nevi

Ruling out inflammation before intervention-

(i)Ashy dermatosis –EDV (?variants)

(ii)LPP (LPP inversus-in folds)

Riehl’s melanosis

Seborrheoic melanosis associated with seb derm-

TCIs

Facial AN-

Velvety

Met+Roz+Octreotide+Pioglit

Hype about IR

Facial melanosis-

Normal

Diffuse (systemic cause)

Focal

Normal demarcation lines-

Follows neuro-axial line of Sherrington

Comes back,even after treatment

Maturational dyschromia

Cushing’s disease

Addison’s disease (even in sun protected areas)

Nelson syndrome

Hemochromatosis

Triad of hyperpigmentation,DM and cirrhosis liver

Hyperthyroidism

Phaeochromocytoma (slow release of beta MSH)

All liver conditions leads to hyperpigmentation

Hyperpigmentation with induration-

Systemic sclerosis

POEMS

PCT

B12 deficiency leading to hyperpigmentation-

Decreased glutathione levels

Chloroquine-

Bleaching of hair while hyperpigmentation of skin

Navarathina oil-

Allyl isothiocyanate (photosensitizer)

Nevus of Hori

Riehl’s melanosis-

Obsolete term

Alternative term is Pigmented contact dermatitis

Peribuccal pigmentation of Brocq

Chikungunya-

Nasal pigmentation

Ochronosis-

Polymerisation HG acid in vivo

Treatment of melasma without steroids-

HQ-Steroid-Retinoids

‘Steroid bridge’

Retinoid –Azelaic acid combination

Mechanism of action of retinoids on AN-

HK is the cause for pigmentation in AN

Retinoids act on the HK

Procedures in facial melanosis-

Q-Yag laser

Biolite (<25,000 Rupees) –Blue for acne

Freshly prepared TCA peel (35%) is the best choice for melasma

Peel IritationApply

No irritationContinue

Irritation-Stop

Very good response with frictional dermatosis

Never do deep peel immediately after triple combination

Wait for some time and try TCA

Q-yag laser-

(+)No down time

(+)ideal for Tatoos

Carbon toning

Freckles and pigment demarcation lines- Never touch with lasers

Volbella filler

LPP-

Omeprazole

Food additives

Azo dyes in towels