Leukoderma and Vitiligo

Prof. Ossama Hussein Roshdy

It is a term given to a wide range of disorders all are characterized by cutaneous

hypopigmentation.

MELANOGENESIS

Melanin is produced from Tyrosine.

TYPES OF Leukoderma

 INFANCY ( Diffuse-CIRCUMSCRIBED).

 CHILDHOOD (Diffuse/ CIRCUMSCRIBED).

 ADULTHOOD (Diffuse/ CIRCUMSCRIBED).

Leukoderma

INFANCY / Diffuse

Leukoderma

INFANCY / CIRCUMSCRIBED

Leukoderma

CHILDHOOD / CIRCUMSCRIBED

Leukoderma

ADULTHOOD

- White hairs.

- Milky white skin.

- Blue-grey eyes.

- 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.

- 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.

- Rare, autosomal dominant or A. recessive.

- Achromia of hair and/or skin.

- Broad nasal root  dystopia canthorum.

- Heterochromia irides.

- Congenital deafness.

- Medial eyebrows hyperplasia.

- Superficial fungal infection.

- Pityriasis versicolor.

- Unsaturated fatty acids oxidation

products  inhibit tyrosinase activity.

Others:
 - Leprosy.

- Pinta.

- Yaws.

- Bejel.

- Secondary stage Syphilis.

- It is a misnomer.

- Usually present at birth.

- Single but may be multiple, circumscribed.

- Rounded, dermatomal or in whorls and streaks.

- It is an autosomal dominant disease.

- Multiple, irregularly scattered(ashleaf appearance).

- 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).

VITILIGO

ETIOLOGY

Genetic element

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).

Vitiligo theories

- 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.

- Melanocyte activity Melanocyte death.

Evidenced by:

Elevated levels of in Melanocytes of

vitiligo lesions may suggest neurogenic control.

HISTOPATHOLOGY

Vitiligo areas

Absent Melanocytes.

• Cultured Melanocytes at lesion’s peripheries

Sometimes  ?? Inflammatory changes at the borders of lesions.

CLINICALLY

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).

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:

A- Autoimmune diseasaes:

- Addison’s disease.

- Thyroid disease.

- Diabetes.

- Alopecia areata.

B- Uveitis.

C- Auditory problems.

D- Vogt-Koyanagi-Harada Syndrome.
TREATMENT

P = Psoralens

UVA = Ultraviolet A

Systemic PUVA

8 methoxy-psoralen 0.5 mg/kg.

Used for extensive vitiligo.

Topical PUVA

8 methoxy-psoralen 0.05- 0.1% solution.

Used in cases with less than 20% total surface area depigmentation.

Mechanism

PUVA increase the size but not the number of Melanosomes.

UV light

Psoralen + DNA photoadducts

(with thymine bases) DNA inhibition

RNA + protein synthesis.

Decreasing the antibodies directed against Melanocytes.

 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- Can be used in children.

2- Can be used in lactating and pregnant women.

3- Can be used in hepatic or kidney dysfunction.

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.

Segmental or localized vitiligo.

Non progressive-inctive disease.

In areas such as:

- Dorsal aspect of fingers.

- Forehead.

- On hair lines.

- Ankles.

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