Mongolian spot vs Ito’s nevus

Pyeongchon Choice Skin Clinic

Hoon Hur M.D., Ph.D.

<허훈약력>

한양의대졸업피부과전문의의학박사GPT개발자OMS-1 parameter 개발자OMS-2 parameter 개발자OMS-3 parameter 개발자OMS-4 parameter 개발자532nm forte therapy for melanocytic nevus개발자unified GPT for tattoo removal 개발자대한임상피부치료연구회(대피연) 정회원대한임상피부치료연구회(대피연) 회장한양의대피부과외래교수평촌초이스피부과대표원장시인/ 수필가

Mongolian spot -clinically, slate gray nevus/ lumbosacral dermal melanocytosis

-histologically, congenital dermal melanocytosis

Birthmark-congenital dermal melanocytosisAlthough melanocytic, is not a true nevus Incidence of 90% in Asian race / 5% in neonate with blond hair Extensive Mongolian spots may be associated with inborn errors of metabolism

ex) ganglioside in GM1 gangliosidosisheparin sulphate in Hurler’s disease

Ill defined area of blue discoloration, up to several centimeters and in lumbosacral regionMay also occur at other sites including the arm , leg and shoulderAffected skin: gray to blue patch or deep blue patch

not thickened, not skin change not the plaque but the patchround, oval and irregular shape

Histopathologic finding-proliferation of spindle-shaped, dendritic melanocytes in the mid dermis or deep dermis- with melanin granules dissecting bundles of dermal collagen -No associated melanophages

1. Clinical types of Mongolian spot(MS)

1)common Mongolian spot-irregular thin blue patches on the lumbosacral area-It will disappear within 10 years

2)deep blue Mongolian spot-round or rectangular deep blue patch on the lumbosacral area-round or rectangular deep blue patch on the extra-lumbosacral area-It may persist during all life-deep blue MS on the common MS: superimposed MS -deep blue MS on the ectopic MS: superimposed MS

3)ectopic Mongolian spot- gray to blue patch or thin blue patch on the extra-lumbosacral area- It will disappear within 10 years

2. Based on the site, types of Mongolian spot

1)common Mongolian spot : lumbosacral area

2)ectopic Mongolian spot: extra-lumbosacral area

3. Based on the color, types of Mongolian spot

1)common Mongolian spot-gray to bluish or thin blue patch-on the lumbosacral area-elongated, spindle-shape d, dendritic melanocytes in the mid dermis-It will disappear within 10 years

2)deep blue Mongolian spot-deep blue patch-on the lumbosacral area or extra-lumbosacral area-elongated, spindle-shape d, dendritic melanocytes in the deep dermis-It may persist during all life

4. Based on the regression , types of Mongolian pot

1)regession type(97%)-common Mongolian spot, ectopic Mongolian spot2)persistent type(3%)-deep blue Mongolian spot, Superimposed MS, Speckled MS

Other variants include the following types:

1)Generalized MS: Involve large areas covering almost entire anterior or posterior trunk andextremities .initial marker : inborn errors of metabolism

2)Aberrant MS(ectopic MS): Involve unusual sites such as head and neck region or extremities

3)Superimposed MS: A darker Mongolian spot overlies a lighter one.

4)Halo-like MS: When café-au-lait macules and melanocytic nevi reside within areas of Mongolian spot, there will be a white rim lacking dermal melanocytes surrounding eachlesion.

5)Speckled MS: Present as groups of dotted deep blue pigmentation on the thin blue patch.

Extensive Mongolian spots will disappear within 10 years because of thin blue patches.Extensive Mongolian spots may be an initial marker of inborn errors of metabolism

Ectopic MS(thin blue patch)will disappear

Common MS(thin blue patch)will disappear

Ectopic Mongolian spots will disappear within 10 years because of thin blue patches

Mongolian spots

1)melanocytes(in upper dermis)--gray to blue patch: disappear

2)melanocytes(in mid dermis)--thin blue patch: disappear

3)melanocytes(in lower dermis)--deep blue patch: persist

A deep blue Mongolian spot is ectopic and may become persistent because of deep blue patch

Mongolian spots

1)melanocytes(in upper dermis)--gray to blue patch: disappear

2)melanocytes(in mid dermis)--thin blue patch: disappear

3)melanocytes(in lower dermis)--deep blue patch: persist

Two deep blue Mongolian spots are on the lumbosacral area and may become persistentbecause of deep blue patch.

The common Mongolian spots are on the lumbosacral area and will disappear within 10 years because of thin blue patches.

Superimposed Mongolian spot: deep blue MS on common MS or deep blue MS on ectopic MS

The speckled Mongolian spot is ectopic and has the groups of dotted deep blue pigmentation on the large thin blue patch.The speckled Mongolian spot may persist into adulthood.GPT is needed.D/Dx: Ito’s nevus-This lesion becomes darker. <대피연제공>

Speckled Mongolian spot(Before treatment)D/Dx: Ito’s nevus-This lesion becomes darker

OMS-2 PTPulse stacking GPT+ sliding-stacking GPT+ sliding GPT

Speckled Mongolian spot(After treatment)

Speckled Mongolian spot를전통적인레이저치료법으로치료시흉터기생길수있기때문에 1- 2주일간격으로 OMS2-PT와 GPT를 50회치료하여 speckled Mongolian spot를 downtime 없이흉터없이치료할수있다.

Halo-like Mongolian spotcafé-au-lait spots reside within areas of Mongolian spot

a white rim lacking dermal melanocytes surrounding Mongolian spot

A 13-year-old girlEctopic Mongolian spot( ill- defined, thin, blue patch on the thigh)Ectopic Mongolian spot will disappear.Treatment: Pulse stacking GPT+ sliding-stacking GPT+ sliding GPT

(loss of protective extracellular fibrous sheathdestruction of dermal melanocytes)

fading: thin blue ectopic MS

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Superimposed Mongolian spot (13-year-old girl)A single oval deep blue MS on the huge thin blue ectopic MSTreatment: Pulse stacking GPT+ sliding-stacking GPT+ sliding GPT

fading: thin blue ectopic MS

slow fading: deep blue MS

<대피연제공>

(loss of protective extracellular fibrous sheathdestruction of dermal melanocytes)

Ectopic MS(thin blue patch on the right lower leg)Finally, a single thin blue ectopic MS will disappear Treatment: Pulse stacking GPT+ sliding-stacking GPT+ sliding GPT

<대피연제공>

Speckled Mongolian spot1.Speckled Mongolian spot is ectopic

and has the groups of dotted deep blue pigmentation on the thin blue patch.

2.Speckled Mongolian spot may persist into adulthood. (fading)

3.Congenital dermal melanocytosis4.Traditional laser therapy or GPT

Ito’s nevus1.The unilateral Ito’s nevus is present on the

right shoulder and right upper arm andhas the groups of dotted deep blue pigmentation on the huge thin blue patch.

2.Ito’s nevus persists during all life. (darkening)

3.Congenital dermal melanocytosis4.Traditional laser therapy or GPT

Differential diagnosis

Differential diagnosis

Pathogenesis of Mongolian spot

Fetal embryo시기에 neural crest cells에서기원하는melanoblasts가 epidermis의 basal layer에서epidermal melanocytes로전환되고또한 hair follicle의 follicular melanocytes로전환되고그나머지melanocytes가 dermis쪽으로 dropping되어 dermal melanocytes로존재하고keratinocytes와 dermal fibroblasts에서분비되는 melanocyte stimulating growth factor가끊임없이dermal melanocytes를자극하고 proliferation시켜 congenital dermal melanocytosis가발생하게된다. 또한 keratinocytes와 dermal fibroblasts에서분비되는 nerve growth factor는melanocyte의nerve growth factor-receptor와반응하여 transdermal melanocyte migration을촉진시켜congenital dermal melanocytosis가발생하는데도움을준다.그리하여현미경학적으로 fetal age 3개월때 dermal melanocytes가존재하고, fetal age 7개월때에는육안으로도 dermal melanocytes를확인할수있다.

mechanism of regression vs mechanism of persistence

< Mongolian spot가 regression되는기전과지속되는기전>

1.dermal melanocytes는자신을보호하기위해서 protective extracellular fibrous sheath에의하여둘러쌓여있는데나이를먹어감에따라 protective extracellular fibrous sheath가소실되어 dermalmelanocytes가파괴되기시작하고, keratinocytes와 dermal fibroblasts에서분비되는 melanocytestimulating growth factor의농도가감소되기시작하면 dermal melanocytes의 proliferation을유지할수없게되어 common Mongolian spot에서는 dermal melanocytes의 regression이일어나게된다.

2.deep blue Mongolian spot의경우에서는나이를먹어감에도 dermal melanocytes를감싸고있는protective extracellular fibrous sheath가소실되지않고지속적으로존재하여 dermal melanocytes가파괴되는것을방해하고,keratinocytes와 dermal fibroblasts에서분비되는melanocyte stimulating growth factor의농도가지속적으로유지되어 dermal melanocytes의proliferation을유도하여성인에서도 deep blue Mongolian spot가소실되지않고지속적으로존재하게된다.

Extensive Mongolian spot in inborn errors of metabolism

<inborn errors of metabolism(IEM)에서 extensive Mongolian spot이발생하는 mechanism>

1)accumulated metabolites(=GM1 gangliosidosis에서는 ganglioside/Hurler's disease에서는heparin sulphate)등이 TrK protein과결합하여metabolite-TrK protein binding이 melanocyte의tyrosine kinase type receptor에반응하여 transdermal melanocyte migration을촉진시킨다.

2)keratinocytes와 dermal fibroblasts는 nerve growth factor를분비하고, inborn errors of metabolism에의하여발생된 metabolites가 nerve growth factor의 activity를증가시키고다시nerve growth factor는melanocyte의 nerve growth factor-receptor와반응하여 transdermalmelanocyte migration을촉진시켜 extensive Mongolian spot가발생하게된다.

3)따라서 extensive Mongolian spot와 multiple Mongolian spot는 inborn errors of metabolism의 initial marker일수있기때문에 extensive Mongolian spot와 multiple Mongolian spot가발생한신생아에서는 inborn errors of metabolism의유무를꼭검사해봐야한다.

4)inborn errors of metabolism에서발생하는 extensive Mongolian spot는성인이되기전에전부소실되기때문에굳이레이저치료를하여제거할필요가없다.

Treatment of Mongolian spot

1)wait for regression: regression type(97%)/ persistent type(3%)

2)traditional laser therapy with downtimeQSNL: 1064nm/3mm/10-12J/2HzRuby laser: 694nm/3mm/6J/1Hz

3)laser therapy without downtimeOMS-2 PT subsequently GPTOMS2-PT: 3mm/5J/10Hz (3-5seconds:pulse stacking)GPT: 7mm/2.2J/10Hz (QX-MAX laser)GPT: 7mm/2.4J/10Hz (spectra laser)

A 5-year-old boyOnset: at birthA single deep blue plaque on the right temple areaThis lesion is darkeningThis lesion is not a patch but a plaque

What is the diagnosis?

<대피연제공>

Congenital blue nevus (patch blue nevusplaque blue nevus: dermatocytoma)A 5-year-old boyOnset: at birthA single deep blue plaque on the right temple areaThis lesion is darkeningThis lesion is not a patch but a plaqueD/Dx: deep blue Mongolian spot is not a plaque but a patch.Treatment: 1064nm/3mm/10J/2Hz+subsequently 532nm/3mm/4J/2Hz

Ophthalmic branch areadeep blue patch

This lesion is darkening

Ito’s nevusUnilateral blue patch on the shoulder and chestDotted deep blue pigmentation on large thin gray to blue patchThis lesion become darker. (D/Dx: Mongolian spot will fade.)1064nm/3mm/10-12J/2Hz (intervals of treatment: 1 month)GPT:1064nm/7mm/2.4J/10Hz (intervals of treatment: 1-2 weeks)

Ito’s nevus

Ito’s nevusUnilateral blue patch on the shoulder and chestDotted deep blue pigmentation on large thin gray to blue patchThis lesion become darker. (D/Dx: Mongolian spot will fade.)1064nm/3mm/10-12J/2Hz (intervals of treatment: 1 month)GPT:1064nm/7mm/2.4J/10Hz (intervals of treatment: 1-2 weeks)

acquired epidermal melanocytosis

: 3%

: 97%

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