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Pigmentation disorders of skin
Hypopigmented
• Symmetrical
• Vitiligo
• Piebaldism (AD)
• Albinism (AR)
• Asymmetrical
• Pityriasis alba / versicolor
• Ash leaf macule
• Nevus anemicus
• Nevus achromicus
• Nevus depigmentosis
Congenital
Pityriasis alba
• Hypopigmented recurrent scaly macule
• In children (6-12 yr) decreases on puberty
• On cheeks and face
• Recurrent and more common in summer
Piebaldism
• Congenital AD
• Localised depigmented macule
with symmetrical involvement of
central forehead, ventral
trunk,upper and lower extremities
with acral sparing
• White forelock
• Wardenberg syndrome =
piebaldism + SNHL
piebaldism
• Characteristically areas of hypopigmentation contain
hyperpigmented or normally pigmented macules or Islands of hyper
pigmentation within the lesions
Albinism (albin is recessive)
• Congenital AR
• Complete absence of melanin
• Albinism should not be considered as a differential diagnosis of
hypopigmented macule/patch as there no patch / macule, instead
there is diffuse hypopigmentation of skin & hair throughout the body.
• Associated eye defects
• Absence of pigment in iris and retina (eye defects are present in all cases)
Nevus achromicus
• u/l Single hypopigmented patch on trunk
or proximal extremity + erythema
response present on massage (unlike
naevus anemicus )
• Limited to single neuronal segment
• Feathered margins
• No leucotrichia (unlike vitiligo )
• Does not disappear on pressure with
glass slide
• Present at birth and static (do not
increase in size )
Nevus anaemicus
Presents since birth and static
• vascular anomaly presents clinically as
hypopigmented patch or macule.
• Pressure on lesion makes the lesion to disappear
and indistinct from surrounding skin.
• Most commonly on the upper chest.
• f. Massage fails to develop erythema (in
contrast to Nevus achromicus)
Nevus achromicus Nevus anemicus
Diascopy Can differentiate fromsurrounding
skin
Cannot differentiate from
surrounding skin
Heat Erythema develops No erythema
Becker nevus
• Irregular pigmentation of
shoulder , upper chest or
scapula region
Vitiligo
• depigmented or nonpigmented nonscaly macule sharply defined
• Acquired
• Associated with AutoImmune d/s
• DM, thyroid d/s (hypothyroidism) alopecia areata, addisons d/s
• koebner phenomenon +
• Can occur anywhere in body (depending on type of vitiligo ~ areas subjected
to repeated friction & trauma are frequently affected, i.e. Dorsum of hands &
feet, knee, elbow)
• Lecotrichia (depigmented hair) poor prognosis
• Lesion on face best prognosis
Vitiligo
• Most cases begin between the age of 10-30 years
• Margins are elevated and hyperpigmented àSalloped hyperpigmented
margins
• Vitiligo
• Vitiligo vulgaris
• Segmental  stable & static course /not associated with AI d/s
• Vitiligo universalis
• Generalised vitiligo
• Lip tip ,tip of penis,vulva and nipples involved
Segmental • u/l along dermatome
• Stable & static course
• Not associated with AI disease
Vitiligo vulgaris • Most common
• b/l symmetrical
Acrofacial
Lip tip • Involvement tip of lip nipple penis finger tips
Vitiligo universalis • Generalised
• a/w AI d/s like DM pernicious anemia hashimotos
ds
• Trichrome vitiligowith three colors (white, light brown, dark brown)
represents different stages of evolution
• Poor prognosis
• Leucotrichia
• Lesion over bony prominences
• Acrofacial type
• Long standing
Treatment
• <20 %
• Topical steroids
• Topical vitamin D analogue
• β FGF cream
• Topical immunomodulators  tacrolimus
• If no response
• PUVA/ PhotoRx
• If no response
• Grafting/ melanocyte transplant
• >20%
• Systemic steroids
• Azathioprine
• Levamisole
• Phototherapy
• Narrow band UVB  radiation of choice
• PUVA
• PUVASOL( PUVA +SOLarlight)
Sx treatment of vitiligo
• Ultra thin partial thickness skin graft
• Autologous melanocytic transfer
• Cultured
• Noncultured
Hyperpigmented lesions
Ceruloderma
• Blue coloured skin
• 3 types
• Nevus of ota
• Nevus of ito
• Mongolian spot
Nevus of ito
• Pigmentation of skin innervated
by lateral branches of
supraclavicular nerve
Nevus of oto
• Oculodermal melanosis
Nevus of ota Nevus of ito
both • u/l
• Bluish grey
• u/l
• Bluish grey
Site Trigeminal nerve (ophthalmic &
maxillary division )face
Acromiocalvicular nerveshoulder
& posterior neck
Malignant potential Rare present with i/Lglaucoma Absent
Rx NdYAG laser /ruby laser (Q
switched mode)
NdYAG laser /ruby laser
Mongolian spot
• Since birth
• Large blue to grey patch
• Buttocks and lumbosacral area
• almost all newborn babies of African and Asian origin
• Mongolian spots are benign skin markings, and are not
associated with any illnesses, complications or risk factors.
• There is no known prevention and they generally fade in a
few years and disappear by puberty. Though occasionally
they persist into adulthood, there is no need for treatment
Café aulait spots
• Neurofibrmatosis (von
recklinghausens d/s)
• Tuberous sclerosis
• MEN
• Albrights syndrome
• Fibrous dysplasia
• Fanconis anemia
• Leopard symfrome
Freckles
• Yellowish / brownish
macules of
hyperpigmentation seen in
exposed parts of skin
Freckles Lentigens
Increased melanin synthesis Increased mealnosomes
Sun exposed areas involved Anywhere
Sunexposure  accentuation No accentuation
a/w xeroderma pigmentosa PJ syndrome GI polyps /peri oral lentigens
Tuberous sclerosis
• Hypopigmented macules ("ash leaf spots'') of tuberous sclerosis are
associated with:-
• a. Shagreen patches (area of thick leathery skin on back & neck)
• b. Facial angiofibroma (adenoma sebaceum)
• c. Cafe au lait spots
Tuberous sclerosis
• Vogts triad
• MR
adenoma sebaceum
• Seizures
Benign neoplasm a/w TS
• Rhadbomyoma of myocardium
• Angiomyoma of
liver,kidney,pancreas
• Ependymoma
• Astrocytoma
Melasma
• Acquired
• a/w pregnancy and OCP
• Brown patch in maxillary and zygomatic
area
• Butterfly rash
• Spare nasolabial fold
• Well demarcated

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Pigmentation disorders of skin dermatology revision notes

  • 2. Hypopigmented • Symmetrical • Vitiligo • Piebaldism (AD) • Albinism (AR) • Asymmetrical • Pityriasis alba / versicolor • Ash leaf macule • Nevus anemicus • Nevus achromicus • Nevus depigmentosis Congenital
  • 3. Pityriasis alba • Hypopigmented recurrent scaly macule • In children (6-12 yr) decreases on puberty • On cheeks and face • Recurrent and more common in summer
  • 4. Piebaldism • Congenital AD • Localised depigmented macule with symmetrical involvement of central forehead, ventral trunk,upper and lower extremities with acral sparing • White forelock • Wardenberg syndrome = piebaldism + SNHL
  • 5. piebaldism • Characteristically areas of hypopigmentation contain hyperpigmented or normally pigmented macules or Islands of hyper pigmentation within the lesions
  • 6. Albinism (albin is recessive) • Congenital AR • Complete absence of melanin • Albinism should not be considered as a differential diagnosis of hypopigmented macule/patch as there no patch / macule, instead there is diffuse hypopigmentation of skin & hair throughout the body. • Associated eye defects • Absence of pigment in iris and retina (eye defects are present in all cases)
  • 7. Nevus achromicus • u/l Single hypopigmented patch on trunk or proximal extremity + erythema response present on massage (unlike naevus anemicus ) • Limited to single neuronal segment • Feathered margins • No leucotrichia (unlike vitiligo ) • Does not disappear on pressure with glass slide • Present at birth and static (do not increase in size )
  • 8. Nevus anaemicus Presents since birth and static • vascular anomaly presents clinically as hypopigmented patch or macule. • Pressure on lesion makes the lesion to disappear and indistinct from surrounding skin. • Most commonly on the upper chest. • f. Massage fails to develop erythema (in contrast to Nevus achromicus)
  • 9. Nevus achromicus Nevus anemicus Diascopy Can differentiate fromsurrounding skin Cannot differentiate from surrounding skin Heat Erythema develops No erythema
  • 10. Becker nevus • Irregular pigmentation of shoulder , upper chest or scapula region
  • 11. Vitiligo • depigmented or nonpigmented nonscaly macule sharply defined • Acquired • Associated with AutoImmune d/s • DM, thyroid d/s (hypothyroidism) alopecia areata, addisons d/s • koebner phenomenon + • Can occur anywhere in body (depending on type of vitiligo ~ areas subjected to repeated friction & trauma are frequently affected, i.e. Dorsum of hands & feet, knee, elbow) • Lecotrichia (depigmented hair) poor prognosis • Lesion on face best prognosis
  • 12. Vitiligo • Most cases begin between the age of 10-30 years • Margins are elevated and hyperpigmented àSalloped hyperpigmented margins • Vitiligo • Vitiligo vulgaris • Segmental  stable & static course /not associated with AI d/s • Vitiligo universalis • Generalised vitiligo • Lip tip ,tip of penis,vulva and nipples involved
  • 13. Segmental • u/l along dermatome • Stable & static course • Not associated with AI disease Vitiligo vulgaris • Most common • b/l symmetrical Acrofacial Lip tip • Involvement tip of lip nipple penis finger tips Vitiligo universalis • Generalised • a/w AI d/s like DM pernicious anemia hashimotos ds
  • 14. • Trichrome vitiligowith three colors (white, light brown, dark brown) represents different stages of evolution
  • 15. • Poor prognosis • Leucotrichia • Lesion over bony prominences • Acrofacial type • Long standing
  • 16. Treatment • <20 % • Topical steroids • Topical vitamin D analogue • β FGF cream • Topical immunomodulators  tacrolimus • If no response • PUVA/ PhotoRx • If no response • Grafting/ melanocyte transplant • >20% • Systemic steroids • Azathioprine • Levamisole • Phototherapy • Narrow band UVB  radiation of choice • PUVA • PUVASOL( PUVA +SOLarlight)
  • 17. Sx treatment of vitiligo • Ultra thin partial thickness skin graft • Autologous melanocytic transfer • Cultured • Noncultured
  • 19. Ceruloderma • Blue coloured skin • 3 types • Nevus of ota • Nevus of ito • Mongolian spot
  • 20. Nevus of ito • Pigmentation of skin innervated by lateral branches of supraclavicular nerve Nevus of oto • Oculodermal melanosis
  • 21. Nevus of ota Nevus of ito both • u/l • Bluish grey • u/l • Bluish grey Site Trigeminal nerve (ophthalmic & maxillary division )face Acromiocalvicular nerveshoulder & posterior neck Malignant potential Rare present with i/Lglaucoma Absent Rx NdYAG laser /ruby laser (Q switched mode) NdYAG laser /ruby laser
  • 22. Mongolian spot • Since birth • Large blue to grey patch • Buttocks and lumbosacral area • almost all newborn babies of African and Asian origin • Mongolian spots are benign skin markings, and are not associated with any illnesses, complications or risk factors. • There is no known prevention and they generally fade in a few years and disappear by puberty. Though occasionally they persist into adulthood, there is no need for treatment
  • 23. Café aulait spots • Neurofibrmatosis (von recklinghausens d/s) • Tuberous sclerosis • MEN • Albrights syndrome • Fibrous dysplasia • Fanconis anemia • Leopard symfrome
  • 24. Freckles • Yellowish / brownish macules of hyperpigmentation seen in exposed parts of skin
  • 25. Freckles Lentigens Increased melanin synthesis Increased mealnosomes Sun exposed areas involved Anywhere Sunexposure  accentuation No accentuation a/w xeroderma pigmentosa PJ syndrome GI polyps /peri oral lentigens
  • 26. Tuberous sclerosis • Hypopigmented macules ("ash leaf spots'') of tuberous sclerosis are associated with:- • a. Shagreen patches (area of thick leathery skin on back & neck) • b. Facial angiofibroma (adenoma sebaceum) • c. Cafe au lait spots
  • 27. Tuberous sclerosis • Vogts triad • MR adenoma sebaceum • Seizures
  • 28. Benign neoplasm a/w TS • Rhadbomyoma of myocardium • Angiomyoma of liver,kidney,pancreas • Ependymoma • Astrocytoma
  • 29. Melasma • Acquired • a/w pregnancy and OCP • Brown patch in maxillary and zygomatic area
  • 30. • Butterfly rash • Spare nasolabial fold • Well demarcated