Ebook Color atlas of cosmetic dermatology (2/E): Part 2

S IX
Vasc u l a r A l te rat i o n s


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CHAPT E R 29

Angio ke rato m a

Angioke ratomas a re te la ngiectasias with keratotic ele­
ments . They present i n d i ffe rent c l i n ical scena rios i n c l u d ­
i n g ( a ) solitary or m u lt i p l e a ngioke ratomas occ urring
p red o m i n a ntly on lower extre m ities; ( b) a ngiokeratoma of
Fordyce affecti n g the sc rotu m a n d the vu lva ; ( c ) a ngiok­
e ratom a of M i be l l i , a n a utoso m a l d o m i n a nt d isorder
affecti n g d o rs u m of h a n d s a n d feet, e l bows, a n d knees;
(d) a ngiokerato ma corporis d iffus u m associated with
Fa bry's d isease, an X- l i n ked recessive d isord e r c h a rac­
terized by a.-ga lactosidase-A d eficie ncy and affecting
the lowe r a bd o m e n , buttoc ks, a n d ge n ita l ia ; a n d ( e )
a ngioke ratoma c i rc u mscri ptu m usua l ly grou ped on one
extre m ity.

E P I D E M I O LOGY
Age: solita ry o r m u ltiple a ngiokeratomas u s u a l l y affect
you n g a d u lts , a ngiokeratomas of Fordyce affect m i d d le­

aged and elderly i n d ivid u a l s . Angioke ratoma of M i be l l i
a n d a ngioke rato ma c i rc u msc r i ptu m a re u s u a l l y d iag­
n osed in c h i l d h ood .

Sex: a ngiokeratoma of M i be l l i a nd a ngioke ratoma c i r­
c u mscri pt u m exh i bit fem a l e pred o m i na nce. Otherwise,
there is no sex pred is position .

PHYS I CAL EXAM I NAT I O N
R ed t o violaceous, we l l - c i rc u m sc r i bed hyperke ratotic
pa p u les a n d p l a q ue s .

A

D I F F E R E N T I A L D I AG N OS ES
Sol ita ry lesions ca n be m ista ken for mela noma , a cq u i red
hemangioma, lym p ha ngio m a , seborrheic ke ratos is, a n d
wa rts .

LABORATORY DATA
• D e r m atopat h o l ogy
M a rked d i lated , t h i n -wa l l ed blood vesse ls in the pa p i l l a ry
d e r m i s , associated with an overlying acanthotic hyperker­
atotic epidermis.

COU RS E MANAG E M ENT

B

M a nagement o f a ngiokeratomas rema i ns a c h a l lenge.

Figure 29. 1 (A) Angiokeratomas on the abdomen of a young patient.

M a n y m od a l ities have been reported i n the l iterature with

(B) Angiokeratoma imaged through an epiluminescence microscope

va riable s uccess . Treatment m od a l ities i n c l u d e

(DermLite)


Sect i o n 6 : Va sc u l a r A l te rat i o n s

•

I

1 69

Lasers : a ngiokeratomas have occasionally been treated
successfu lly with lasers.
- The p u lsed dye laser ( P OL) is an effective d evice for
the i m provement of the vasc u l a r component of
a ngiokeratomas,

but

freq uently

some

keratosis

rema i n s . The target c h romophore is hemogl o b i n .
P O L has proven successful a t 595 n m , 5-to-7- m m

spot, 9 t o 1 1 J/c m 2 , O C O 30/20. Cove ring the a ngiok­
e rato m a with a glass s l i d e , that is, d iascopy, is h e l p­

fu l . The end point is lesional p u r p u ra . H ea l i ng occ u rs
in more than 10 to 14 days. M u lt i p l e treatments may
be req u i red ( Fig. 29 . 3 ) .
- Res u rfacing lasers s u c h as C0 2 and Er:YAG lasers ca n
be uti l ized for lesiona l va porizatio n . Patients genera l ly
req u i re local i nfi ltration with 1 % l id oca i n e with or with­
out epinephrine prior to treatment. The U ltra Pu lse C0 2
( Lu men is, Sa nta Clara, CAl is employed using a 3-m m
col l i mated hand piece, with an energy of 300 to 500 mJ
with nonoverlapping pu lses . The va rious sca n ned C0 2
lasers such as the Sharplan FeatherTouch a re

Figure 29.2 Angiokeratoma on the left thigh resistant to m ultiple treat­

ments with pulsed dye laser

em ployed using the 1 25-m m hand piece, 3-m m sca n
size at 14 to 40 W. The treatment end point is a blation
to

achieve

lesional

flattening

and

opalescence.

Treatment sites should be clea nsed with sa l i ne soa ked
ga uze

between

laser

passes.

Postoperative

care

req u i res twice d a i ly wash i ng with soa p and water a n d
a ppl ication o f a n a nti biotic oi ntment. Hea l ing occ u rs i n
more t h a n 2 t o 6 weeks. A s with a l l a blative proced u res, sca rring may be observed .
- Other lasers that have been used i n the past with
va riable success i n c l u d e potass i u m -tita nyl-phosphate
laser, a rgon laser, a n d copper va por lase r. Long­
pu lsed N d : YAG ( 1 , 064 n m ) laser has been shown to

be effective in i m prov i n g a ngioke ratomas d u e to its
selectivity a nd its deeper penetration i nto the ski n .
Successfu l treatment with a d ua l -wave length laser

A

system (595 a n d
reported

1 , 064 n m ) has been rece ntly
( Cynergy with M u lti plex™ , Cynosu re,

Westford , MA, U S A ) .
•

O t h e r s u rgical treatments i n c l u d e excision , electro­
ca utery, electrofu lgu ratio n , or c ryosu rgery.

P I T FALLS TO AVO I D
•

Patients s h o u l d be advised that the P O L treatment wi l l
cause o bvious b r u i s i n g for u p t o 14 days.

•

Keratotic

featu res

may

persist

after

treatment.

I m provement is often el usive.

B I B L I OG RAPHY
Gorse SJ , J a mes W , M u rison M S . S u ccessful treatment of
a ngioke ratoma with potass i u m tita nyl phosphate laser. Br
J Dermatol. 2004; 1 50 ( 3 ) : 620-622.

B

Figure 29.3 (A) Biopsy-proven angiokeratoma on the thigh of a young

child. (B) Some resolution after one treatment with pulsed dye laser at a
wavelength of 595 nm with a 1 0-mm spot, pulse duration of 1 . 5 ms, a
fluence of 7. 5 J/cm2 , and DCD 30120


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Color Atlas of Cosmetic Dermatology

La pi ns J , Emtesta m L, M a rcusson J A . Angiokeratomas i n
Fa bry's d isease a n d Fordyce's d i sease : Successful treat­
ment with copper va pour laser. Acta Derm Venereal.
1 993; 73 ( 2 ) : 1 33- 1 3 5 .
Occella C , B l e i d l D , R a m p i n i P, Schiazza L, R a m p i n i E.
Argon laser treatment of c uta neous m u lt i p l e a ngioker­
atomas. Dermatol Surg. 1995;2 1 ( 2 ) : 1 70- 1 7 2 .
Ozd e m i r M , Baysa l I , Engi n B , Ozd e m i r S . Treatment of
a ngiokeratoma of Fordyce with long- p u lse neodym i u m­
d o ped ytt r i u m a l u m i n i u m garnet laser. Dermatol Surg.
2009;35( 1 ) : 92-97 .
Pfi rrma n n G , R a u l i n C , Ka rsa i S . Angioke rato ma o f the
lower extre m ities: Successfu l treatment with a d ua l ­
wavele ngth laser system ( 595 a n d 1 064 n m ) . Eur Acad
Dermatol Venereal. 2009;23( 2 ) : 1 86- 187.
Sommer S , M e rc h a nt WJ , Shee h a n - Da re R . Severe p re­
d o m i n a ntly acra l va riant of angiokeratoma of M i be l l i :
Response t o long-pu lse N d : YAG ( 1 064 n m ) laser treat­
ment. JAmAcad Dermatol. 200 1 ;45 ( 5 ) : 764-766 .

CHAPT E R 3 0

Che r ry a nd Spid e r Angio mas

Cherry a ngiomas, a lso known a s r u by spots, se n i l e
hema ngiomas,

a cq u i red

ca p i l lary

hemangioma,

and

Ca m p bell d e Morga n spots a re very c o m m o n benign vas­
c u l a r lesions that pred o m i n a ntly affect the tru n k . Spider
a ngiomas, a lso known as nevus a ra n eus, spider telangiec­
tasia, a rteri a l spid er, and vasc u l a r spid er, re present loca l­
ized

telangiectasias

rad iating

from

centra l

feed ing

a rterioles. They a re common vasc u l a r lesions that pre­
d o m i n a ntly affect the face, u pper tru n k , a rms, and hands.

EPI OEM I O LOGY
Incidence: very common
Age: cherry a ngiomas-m i d d l e-aged a n d elderly peo ple;
s p i d e r a ngiomas-a l l ages
Sex: more common in fema les
Precipitating factors: cherry a ngiomas can e r u pt d u ri n g

p regnancy or w i t h h e patic d i sease. S pider a ngiomas a re
strongly associated with pregna n cy, i nta ke of ora l contra­
ceptive p i l ls, a n d h e patoce l l u l a r d isease

PATHOG EN ES I S
U n known for both . Assoc iation with pregna n cy, o ra l con­
traceptive use, a n d l iver d isease suggest a hormona l ly
med iated a ngioge n i c mecha n is m .


Sect i o n 6: Va sc u l a r A l te rat i o n s

I

171

PHYS I CAL EXAM I NAT I O N
Cherry a ngioma prese nts as a 1 -to-3-m m bright red to
violaceous,

s mooth ,

d o m e-sha ped

pa p u l e .

Spider

a ngioma d is plays a network o f d i l ated ca p i l l a ries rad iati ng
from a ce ntra l vessel . B oth may bleed when tra u matized .

PATHOLOGY
Che rry a ngiomas show loss of rete ridges as we l l as con­
gested and ectatic ca p i l l a ries a n d postca p i l l a ry ven u les in
the pa p i l la ry dermis. S p i d e r a ngiomas revea l a centra l
asce n d i ng a rte riole that b ra nc hes a n d co m m u n icates
with m u lt i p l e d i lated c a p i l l a ries.

D I F F E R E N T I AL D I AG N OS ES
Cherry a ngiomas ca n be m ista ken for angiokerato m a ,
glomeruloid

hema ngioma ,

pyoge n i c

gra n u l o m a ,

and

n od u l a r mela noma . S p i d e r a ngiomas can be m i sta ken for
genera l i zed essentia l te langi ectasias a n d h ered ita ry h em ­
orrhagic tela ngiectasia .

CO U RS E
Che rry a nd spider a ngiomas a ri s i n g d u ri n g pregnancy
may regress postpa rt u m . S p i d e r a ngiomas a rising i n
c h i l d hood m a y a lso resolve sponta neous ly. Otherwise,
both lesions ten d to persist.

A

MANAG E M ENT
Although

med ica l l y

i nsign ifica nt,

c h e rry a n d

spider

a ngiomas a re freq u e ntly treated for cosmetic p u r poses .
M u ltiple

effective

s u rgica l

treatment

o ptions

exist.

Depend i ng on the proced u re selected , the cost to the
patient

may

va ry

sign ificantly.

Che rry

and

spider

a ngiomas that present d u ri ng pregnancy s h o u l d n ot be
treated u ntil seve ra l months after d e l ivery as they may
resolve on their own .
•

El ectrosu rgery
- El ectrod essication with coagulation ( monopolar set­
ti ng, 1-2 W fol l owed by gentle c u rettage with end­
point of lesional flatte n i ng a n d h em ostas is) has been
the trad itiona l treatment m od a l ity for th ese lesions.
- I t is effective and easi l y a ccess i b l e .
- The potential f o r sca r formation m ust b e considered .

•

Laser su rgery : d ifferent lasers have been used su ccess­
fu l ly in treatment of c h e rry a n d spider angiomas.

B

- P u l sed dye laser ( P OL) is the treatm e nt of c h oice. A

Figure 30. 1 (A) Spider angioma, right nose. (B) Full resolution of spider
angioma after a single pulsed dye laser treatment to central vessel and
surrounding skin

s pot size s h o u l d be selected that matc h es d ia meter
of the a ngioma . With spider a ngiomas, the ce ntra l


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Color Atlas of Cosmetic Dermatology

feed i n g vessel as we l l as the s u r ro u n d i n g vessels
s h o u l d be treated . It is best to com press the lesion
with a m i c roscope s l i d e to b l a n c h all but the centra l
fee d i n g vesse l . A p u r p u r i c laser pu lse s h o u l d be
d e l ivered . The m i c roscope s l i d e shou ld be rem oved
to a l low for coo l i n g of the a rea . S u bseq uently, a p u r­
p u r i c laser p u lse ca n be e m p l oyed to target the
te la ngiectasias rad iating from the feed i n g vesse l . The
p u r p u ric treatment end point re presents coagu lation
of the targeted vessels ( Figs . 30. 1 and 3 0 . 2 ) .
- The potass i u m -tita nyl-phosphate ( KT P ) 532-n m laser
prod u ces a favora b l e res ponse. S pot size s h o u l d
match the lesion d i a m eter. The vessels shou l d b e

traced out c o m p l etely for m ost effective treatment.
Treatment end point is lesional cleara nce or su perfi­
c i a l white n i ng. E rythema ca n be expected posttreat­
ment, last i n g 24 to 48 h o u rs .

A

- Ca rbon d ioxid e laser ( U itra P u lse 3-m m co l l i m ated
h a n d piece,

300-400

mJ/pu lse,

nonoverlapping

p u l ses; Sharplan FeatherTou ch 1 25- m m h a n d piece,
14-40 W, 3-mm sca n size, nonoverla p p i n g p u lses)
has been e m p l oyed as secon d-l i n e thera py with
su ccess . Treatment e n d po i n t is lesional flatte n i n g .
Potentia l sca r formation m ust be consid ered .
•

Light thera py
- I ntense p u l sed l ight ( I P L) has a lso been e m p l oyed
with some su ccess. As coagu lation is needed fo r
lesional reso l ut i o n , h igher fluences may be req u i red
for treatm ent efficacy.

•

S u rgical exc ision
- Excision should be reserved for lesions that a re resis­
ta nt to other treatments. A posto perative sca r is
expected w h i c h may be less cosmetically pleasing
t h a n the a ngioma .

P I T FALLS TO AVO I D
•

B

Figure 30.2 (A) Cherry angiomas on the trunk in a middle-aged female.
(B) The appropriate endpoint is purpura obtained after pulsed dye laser

treatment (wavelength of 595 nm, 7-mm spot. 1 . 5-ms pulse duration,
f/uence of 1 2 J/cm 2 , DCD 30120)

Patie nts need to be cou nseled as to the l i ke l i h ood of
o bvious p u r p u ra fo l l owi n g treatment with P D L that may
persist for 1 0 to 14 d ays , espec i a l l y off the face. Lesions
a re less l i kely to be com pletely treated at s u b p u r p u ric
fluences.

•

S i m ple electrocautery may be j u st as effective as P D L
at a red uced cost t o t h e patient.

•

Com press i n g the lesion with a glass slide d u ri n g PDL o r
K T P treatment is h e l pful t o m i n i mize its s i z e a n d a l low­
i ng for greate r laser penetrati o n . This red u ces the tota l
energy needed for coagu lation a n d i n c reases the treat­
ment success rate .

•

M u lt i p l e treatme nts may be req u i red , in pa rti c u l a r for
la rge spider a ngiomas.

A

Figure 30.3 (A) Cherry angioma, chest.


Sect i o n 6 : Va sc u l a r A l te rat i o n s

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1 73

B I B L I OG RAPHY
Dawn G , G u pta G . Com pa rison o f potass i u m tita nyl p h os­
p hate vasc u l a r laser a n d hyfrecato r in the treatment of
vasc u l a r

spiders

and

che rry

a ngiomas.

Clin

Exp

Dermatol. 2003 ; 28(6) : 58 1 -583 .
Fod or L, R a m o n Y, Fodo r A, Ca r m i N , Peled I J , U l l ma n n
Y. A side- by-side pros pective study o f i ntense p u l sed l ight
and N d : YAG laser treatment fo r vasc u l a r lesions. Ann

Plast Surg. 2006; 56(2 } : 1 64- 1 70 .

B

c

D

Figure 30.3 (ContinuedJ (B) Pulsed dye laser treatment to cherry angioma

utilizing diascopy (C) Purpura immediately post pulsed dye laser treat­
ment. (D) Complete resolution of cherry angioma after one pulsed dye
laser treatment

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Color Atlas of Cosmetic Dermatology

CHAPT E R 3 1

G ra nu l o m a Facia l e

G ra n u loma fac i a l e ( G F ) was fi rst d escri bed by Wigley i n
1 945 w h o la beled t h e d i sease "eos i n o p h i l ic gra n u l o ma . "
P i n kus re n a m ed this d isorder gra n u loma fac i a l e i n 1952.
G F is a n i d i o pathic c h ro n i c c uta neous d isorder that usu­
a l ly i nvolves the face, pa rt i c u l a rly the nose . It ca n prese nt
with a si ngle lesion or m u ltiple lesions.

E P I D E M I O LOGY
Incidence: u n c o m m o n
Age: 30 t o 50 yea rs
Race: pri m a ri ly seen in Caucasians
Sex: ma les > fem a l es

Figure 3 1 . 1 Granuloma faciale on the scalp

PATH OG E N ES I S
U n k nown , but may b e mediated b y i m m u ne c o m p lex
d e position .

PHYS I CAL EXAM I NAT I O N

Si ngle i n d u rated facial brown ish-red pa pule o r plaque.
Some lesions may have telangiectasia . M u ltiple lesions may
be present. Extrafacial sites rarely observed . Lesions may
vary in size from m i l l i meters to centimeters ( Fig. 3 1 . 1 ) .

D I FFERENTIAL D I AG N OS ES
Cutaneous l u pus erythematos us, sa rco idosis, lym p h o m a ,
pseudolym phoma , c uta neous T-ce l l

lym p h o m a , fixed

d ru g e r u pti o n , rosacea .

D E R M ATOPATHOLOGY
Dense, polymorphous i nflam matory cell i nfi ltrate i n the
u pper two-t h i rds of the dermis. The i nfi ltrate is com posed
of n u merous eosinoph i ls, neutrophi ls, lym phocytes, a n d
h istiocytes . A pro m i nent grenz zone is c h a racteristica lly
present. Leu kocytoclastic vasc u l itis is freq uently observed .

CO U RS E
The lesions of G F a re usua l ly c h ro n i c a n d o n l y occasion­
a l ly resolve s ponta neously.


Sect i o n 6 : Va sc u l a r A l te rat i o n s

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1 75

MANAG E M ENT
Difficu lt t o treat with a ny modal ity. A n y s uccessfu l treat­
ment often leaves sca rring.

• To p i c a l Treat m e n t
•

Corticosteroids: topica l , i ntra lesio n a l

•

Tac ro l i m u s o i ntment (0. 1 % )

• Syste m i c Treat m e n t
•

Da psone

•

Anti m a l a ri a l s

•

Colc h ic i n e

•

Cl ofaz i m i n e

•

G o l d i nj ecti ons

A

S U RG I CAL TREAT M E N T
•

C ryos u rgery:

m u ltiple

reports

i n d icati ng su ccessful

c l ea ra n c e . Resu lts a re u n pred icta ble ( Fig. 3 1 . 2 ) .
•

S u rgical excision .

•

Derm a b rasion .

•

El ectrosu rgery.

• L i g h t Treat m e n t
•

Topica l psora len a n d u l traviolet A ( P UVA) rad iation
thera py

•

Laser thera py: d ifferent lasers have been used in the
treatment of GF with p ro m i s in g resu lts, either as an
a b lative thera py with ca rbon d i oxid e laser o r as a selec­
tive thera py ta rget i n g the prom i n ent vasc u latu re in G F
lesions using the Q-switc hed a rgon laser, p u lsed dye,
d i ode laser, and potass i u m tita nyl phosphate ( KT P )
532-nm l a s e r ( F ig. 3 1 .3 ) .

P I T FALLS T O AVO I D
•

G F is often reca lc itra nt to thera py. Patie nts s h o u l d be
cou nseled that successfu l treatment is often el usive.

B I B L I OG RAPHY
A m m i rati CT, H ruza GJ . Treatment o f gra n u l o m a fac i a l e
w i t h the 585- n m p u l sed d y e laser.

Arch Dermatol.

1 999; 135(8) :903-905.

Apfel berg DB, Dru ker D , Maser M R , Las h H, S pence B
J r, Denea u D. G ra n u l o m a fac i a l e . Treatment with the
a rgon laser. Arch Dermatol. 1 983 ; 1 1 9 ( 7 ) : 573-576.

B

Figure 3 1 .2 (A) Multiple lesions of granuloma faciale on the face. (8) No
significant improvement detected after one treatment with cryotherapy on
a 4-month follow-up visit


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Color Atlas of Cosmetic Dermatology

Chatrath V, R o h rer TE. G ra n u loma fac i a l e successfu l l y
treated w i t h long-pu lsed t u n a b l e d y e laser. Dermatol

Surg. 2002 ;28( 6 ) : 527-529 .
Elston O M . Treatment of gra n u loma fac i a l e with the
p u l sed dye laser. Cutis. 2000;65(2 ) : 9 7-98.
Khaled A , J ones M, Zerma n i R, et a l . G ra n u loma fac i a l e .

Pathologica. 2007 ;99( 5 ) : 306-308.
M a i l l a rd H, G rogna rd C , Toled a n o C, J a n V, Mac het L,
Va i l la nt L. G ra n u l o m a fac i a l e : Efficacy of c ryosu rgery i n
2 cases. Ann Dermatol Venereal. 2000; 1 2 7 0 ) : 77-79 .
To mson N , Ste rl i ng J C , Sa lva ry I . G ra n u loma fac i a l e

treated successfu l l y w i t h topica l tac ro l i m us . Clin Exp

Dermatol. 2009;34(3) :424-42 5 .
Wheela nd R G , Ash l ey J R , S m ith O A , E l l i s O L, Wheela n d
O N . Ca rbon d ioxid e l a s e r treatment o f gra n u loma fac i a l e .

J Dermatol Surg Oneal. 1 984; 1 0 ( 9 ) : 730-733 .

A

B

Figure 3 1 .3 (A) Indurated brownish-red plaque on the left cheek of a
middle-aged female with granuloma facia/e. (B) Two-year follow-up show­
ing resolution of granuloma faciale after m ultiple pulsed dye laser treat­
ments


Sect i o n 6: Va sc u l a r A l te rat i o n s

CHAPT E R 3 2

I

1 77

I nfa ntile H e m a ngio m a

I nfa nti le hema ngioma ( I H l , a lso known as strawberry,
ca p i l l a ry,

or

cavernous

hema ngiom a ,

is

a

benign

e n d oth e l i a l prol iferation that re presents the most com­
mon tumor i n i nfa ncy. I t ca n be c lassified i nto su perfic i a l
hema ngioma ( S H , 55% o f cases ) , deep hema ngioma
( D H , 30% of cases ) , and m ixed su perfi c i a l and deep
hema ngioma ( M H , 1 5% of cases ) . They occ u r m ost com­
m o n ly o n head a n d neck a reas .

EPI D E M I O LOGY
Incidence: 1% to 3 % a re p resent at b i rt h , 10% to 1 2 %
a re p resent b y 1 yea r o f age

Age: majority (80 % ) become a p pa rent between 2 a n d
5 weeks o f age; 2 0 % a re n oted at b i rt h .

Sex: fe ma les a re affected two t o fou r ti mes more t h a n
m a l es

A

Precipitating factors: prematu re i nfa nts a re more com­
monly affected

PHYS I CAL EXA M I NAT I O N
The a p pearance depends o n t h e d e pth o f the heman­
gioma a n d the phase of evol utio n . S H p resents as bright
red -colored p l a q u e . D H presents as a soft dermal o r s u b­
c uta neous nod u l e with a b l u ish- p u r p l e col or. M H shows
featu res of both SH a n d D H . M u lt i p l e truncal heman­
giomas

may

be

o bserved .

I nvol uting

hema ngiomas

demonstrate a flatter su rfa ce with a grayis h - p u r p l e h u e
t h a t begi ns ce ntra l l y a n d expa n d s outwa rd . The h e m a n ­
giomas

m ight

become

u lcerated

and

he morrhag i c .

Resi d u a l fatty tissue, atrop hy, tela ngiecta s i a , s c a r forma­
tion , and hypertrophy may be observed .

B

D I F F E R E N T I AL D I AG N OS ES
Congen ita l hema ngiomas ca n be confused with a vasc u ­
lar

ma lformation

such

as

port-wi n e sta i n

at

b i rt h .

H ema ngiomas a re ge nera l ly present after b i rth versus
vasc u l a r ma lformations, which a re genera l l y present at

b i rth .

LABO RATORY TESTS
• D e r m at o p at h o l ogy
Prol iferations of p l u m p e n d oth e l i a l cel ls that may exte n d
fro m the su perfi c i a l d e r m i s t o the deep su bcuta neous
tiss u e , d e pen d i ng o n the hem a ngioma s u btype.

Figure 32. 1 (A) Left upper eyelid hemangioma in its early growth phase,
a lesion that may threaten the child 's vision. (B) Marked lightening and
flattening of the hemangioma after m ultiple pulsed dye laser treatments


1 78

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Color Atlas of Cosmetic Dermatology

• A n c i l l a ry Tests
•

A n a bd o m i n a l u ltraso u n d s h o u l d be o bta i ned if m o re
t h a n fo u r tru ncal hema ngiomas a re noted prior to
4 months of age .

•

An electroca rd iogra m ( ECG) a n d a ca rd iac EC H O should
be considered for a n y concern of h igh ca rd iac output.

COU RS E
H ema ngiomas c h a racteristica l l y exh i bit th ree phases of
evol ution : ( a ) prol iferative phase, ( b ) i nvol uting phase,
and (c) i nvo l uted phase. The prol iferati ng phase is c h a r­
a cterized by a ra p i d growth p hase that starts at 1 to
2 m o nths of age a n d lasts u nt i l 6 to 9 months of age. This
growth phase is fol l owed by the i nvol uting phase that
usua l l y starts i n the second yea r of l i fe a n d persists for

A

severa l yea rs. M ore than 90% of u ntreated hema ngiomas
i nvol ute, that is, atta i n maxi m a l regression by 9 yea rs of
age. U p to 30% of hema ngiomas leave posti nvol ution
cha nges i n c l u d ing hypopigme ntati o n , sca rring, tela ngiectasi a , and fi b rofatty tiss u e .

COM P L I CAT I O N S
B leed i n g a n d u lceratio n with seco n d a ry i nfection a n d
sca rring, espec ia l ly i n hema ngiomas i nvolvi ng t h e d i a pe r
a rea , a re c o m m o n l y see n . Oth er serious com pl ications
i n c l u d e orbital o bstruction and a m b lyo pia with periorbita l
hema ngiomas, u pper a i rway o bstruction with h e m a n ­
g i o m a s i n the bea rd d istri bution , s p i n a l a bnorma l ities
with l u m bosacra l hema ngiomas, posterior fossa ma lfor­
mation in la rge fac i a l hema ngioma ( P H A C E syn d rome) ,
a n d h igh output c a rd ia c fa i l u re with m u lt i p l e c uta neous
hema ngiomas assoc iated with viscera l i nvolvement.

B

Figure 32.2 (A) Hemangioma on the left fifth toe pad, a location that

in terfered with the child's ability to ambulate. (B) Significant clearing and
near resolution of the hemangioma after multiple pulsed dye laser treat­
ments

KEY CO N S U LTAT I V E QU EST I O N S
•

Onset o f lesion

•

N u m ber of lesions noted

•

U l ceration n oted

•

B l eed i ng noted

•

Prior treatm ents a n d res ponse

MANAG E M E N T
T h e treatment o f I H s is controve rsia l . G iven t h e natu ra l

cou rse o f I H with sponta neous reso l ution, m a n y physi­
cians c h oose to ca refu l ly o bserve the a rea with no
i ntervention, espec i a l l y i n nonfacia l , sma l l , a n d u ncom­
p l icated

hema ngiomas.

Ea rly i ntervention

is recom­

m e n d ed for ( a ) all I H s that i nterfere with the function of
vita l

orga ns

(eg,

periorbita l

hema ngiomas,

a i rway

o bstruction with hema ngiomas i n the bea rd d istr i b ution,


Sect i o n 6 : Va sc u l a r A l te rat i o n s

I

1 79

h igh-output cardiac fa i l u re ) ; ( b ) la rge facia l hema ngiomas
that usua l ly i nvo l ute with permanent d i sfiguri ng; (c) u l cer­
ated hema ngiomas; and (d) hema ngiomas in the d ia per
a rea that a re very l i kely to u lcerate causing severe pa i n .
•

Medica l treatment
- Steroids i n c l u d i ng topica l steroid a pp l i cation ( c lass 1
corticoste roid a p pl ied twice d a i ly with mon itoring
every 2 wee ks) , i ntra lesiona l steroids (tria m c i nolone
a ceto n i d e 1 0 mg!m L a d m i n istered monthly), and oral
steroids ( 1 . 5-2 mg/kg/d of pred n isone) a re the m a i n ­
stay o f treatment. Patie nts m ust be mon itored c l osely,
espec ia l ly with oral steroid use given the risk of sys­
temic com p l ications i nc l u d i ng growth reta rdation a n d
g l u cose a lterations. Loca l ized side effects i n c l u d e
atrophy a n d yeast infect i o n .
- Other treatment options i nc l u d e to pica l i m i q u i mod
( a p p l ied d a i ly ) , i nterferon-a (3 m i l l ion u n its/m 2/d ,

A

S C ) , a nd v i n c ristine (0.05 mg/kg/d if less than 10 kg,
IV ), espec ia l ly in steroid-resista nt I H . As i nterferon-a
is associated with spastic d i plegi a , patients m u st be
mon itored c l osely.
•

P ro p ra nolol at a d ose of 2 mg/kg/d has been recently
reported to be ve ry effective i n treating severe I H s , even
in steroid-resista nt I H s . T h i s treatment is proposed to
re place ora l or i ntravenous steroids that a re associated
with sign ifica nt side effects. H owever, patients on p ro­
pra n olol s h o u l d be c l osely m o n itored for bradyca rd i a ,
hypotension , a n d hypoglycemia espec ia l ly a t the o nset
of the treatment.

•

Laser treatment
- P u lsed dye laser ( P D U treatment i n d u ces sign ifi­
ca ntly faster regression of the I H . Fl u e nces lower
than those of PWS a re effective and a re assoc iated
with lowe r risk of laser- i n d u ced sca rri ng ( Figs . 3 2 . 1 ,
3 2 . 2 a n d 3 2 . 3 ) . P D L has been used exte nsively i n

B

the treatment of I H i n th ree c l i n ical scena rios:

Figure 32.3 {A) Segmental hemangioma in volving the hand of a 1 -year­

1. U l cerated hema ngiomas res pond effectively to
P D L. PDL ma rked ly dec reases the associated
pa i n a n d i n d uces ra pid hea l i ng of the u l ceration
(75% with i n 2 weeks) ( Fig. 32.4) . Res i d u a l sca r
fo rmation from the u l ce ration is expected .

2. S H s c a n respond wel l to P D L if sta rted either
before

or

early

in

the

prol ife rative

phase.

M u ltiple treatments, every 4 to 6 weeks, a re
req u i red in the prol iferative phase. T h e o n ly
exception is a ra pid ly prol ife rating fa c i a l hema n­
gioma . P D L treatment may i n d uce u lceratio n of
these va ria nts so treatm ent s h o u l d be avoided .
I H with deeper components ( M H , D H J res pond
less effectively to PDL beca use of the l i m itation
of penetration of PDL to 1 . 2 mm i n the ski n .
3 . P D L ca n h e l p treat the res i d u a l erythema a n d
tela ngiectasias o n
hemangiomas.

the

s u rface o f i nvol uted

old girl. {B) Complete resolution of the hemangioma after four treatments
with 595-nm pulsed dye laser at low fluences


1 80

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Color Atlas of Cosmetic Dermatology

- Long-pu lsed N d : YAG lasers a re usefu l for photocoagu­
lation of D H s but have a h igher incidence of sca rring.
•

Other

interventions

include

s u rgical

debulking

and

em bol ization . The risks and benefits of each s u rgica l
a pproach should be considered ca refu l ly before i nterven­
tion since the sca r from spontaneous regression is usua l ly

better than the surgica l scar. Em bol ization is uti l ized in
hema ngiomas associated with h igh-output ca rd iac fa i l u re.

P I T FALLS TO AVO I D
•

Use of excessive P O L fluences without s k i n coo l i ng ca n
cause sca r.

•

Pa rents a re u nd ersta n d a bly a nxious a bout their c h i l d 's
hema ngioma . A f u l l d iscussion of the natu ra l c o u rse of

A

hema ngiomas is m a ndatory prior to sta rt i n g thera py.
The option of foregoi n g treatm ent a n d c l i n ica l l y m o n i ­
toring a patient s h o u l d b e reviewed ca refu l ly p r i o r to
sta rt i n g treatment.
•

Pa rents s h o u l d a lso have a rea l i stic idea of the l i m ita­
tions of thera py. La rge hema ngiomas res pond less suc­
cessfu l ly

to

o ra l ,

s u rgica l ,

and

laser

thera py.

C o m p l icated hema ngiomas that may i n te rfere with the
c h i l d 's health s h o u l d be referred to an a p p ropriate
ped iatric spec i a l i st. P a re nts m ust be awa re that treat­
ment wi l l provide an i m provement but may n ot res u lt i n
fu l l resol ution o f t h e h e m a ngioma .
•

Parents n eed to be ed ucated on proper wou n d care,
espec i a l ly for u lcerated hema ngiomas, i n order to
i m prove the c h i l d 's q u a l ity of l ife .

•

F i b rofatty c h a n ges a re ofte n a seq uela of resolved
hema ngiomas.

Such

c h a nges

can

be

B

i m p roved

sign ificantly with n o n a b l ative a n d a blative fract i o n a l
resu rfa c i ng.

B I B L I OG RAPHY
Batta K, G oodyea r H M , M oss C, Wi l l i a m s H C , H i l ler L,
Waters R. R a n d o m ised control led study of early p u lsed
dye laser treatment of u ncompl icated c h i l d hood haeman­
giomas: Resu lts of a 1 -yea r a na lysis.

Lancet 2002 ;

360(9332 ) : 5 2 1 -527 .
Lea ute-La breze C, Du mas de Ia Roq ue E, H u biche T,
Bora levi F, Tha m bo J - B , Ta·leb A. Propranolol for severe
hema ngiomas of i n fa n cy. N Eng! J Med. 2008;358: 2649265 1 .

c

L i YC, McCa h a n E , R owe N A , M a rt i n PA, Wilcsek G A ,

Figure 32.4 (A) Ulcerated hemangioma, isolated nodular type, extremely
painful and hemorrhaging, treated twice with pulsed dye laser 6 Jlcm 2 ,
7-mm spot size, 590 nm. (B) At 2 months ' follow-up, significant healing
of the ulceration after a single treatment with pulsed dye laser. (C) Four

months after initial pulsed dye laser treatment and 2 months after
second pulsed dye laser treatment, there is complete healing of the
ulceration

M a rt i n FJ . S uccessfu l treatment o f i nfa nti le h a e m a n ­
g i o m a s o f the o r b i t w i t h pro p ra n olol . Clin Experiment

Ophthalmol. 2010;38(6): 5 54-559 .
More l l i J G , Ta n OT, Yoh n J J , Weston WL. Treatment of
u l cerated hema ngiomas i nfa n cy. Arch Pediatr Ado/esc

Med. 1 994; 148( 1 0) : 1 1 04- 1 1 0 5 .


Sect i o n 6: Va sc u l a r A l te rat i o n s

CHAPT E R 33

I

1 81

Ke ratosis Pi l a ris At rophica ns

Ke ratosis p i l a ris atro p h ica ns ( K PA) is a gro u p o f i n he rited
d i so rd e rs with th ree su btypes i n c l u d i ng (a) keratosis
p i l a ris atro p h i ca n s fac i e i ( KPAF ) , (b) atrophoderma ver­
m ic u latu m (AV ) , a n d (c) ke ratosis fo l l i c u l a ris s p i n u losa
d ecalva n s ( KFS D ) . KPA F a n d AV present m a i n ly on the
face with K FS D often a p pea r i n g o n the eye b row a n d AV

m ost com m o n l y seen on the c heeks, sparing the eye­
brows a n d sca l p . KFSD can affect the face, sca l p , a n d
tru n k . I n herita nce pattern can b e a utosom a l d o m i na nt
( KPAF, AV) , recessive (AV ) , or X-l i n ked ( KFS D ) .

EPI D E M I O LOGY
Incidence: very ra re; KPAF is the m ost c o m m o n su btype
Age: KPAF a n d KFSD in i nfa ncy; AV in c h i l d h ood
Sex: ma les a re more seve rely affected in KFSD

Figure 33. 1 Keratosis pilaris: fine, sandpaper-like follicular papules on

PATH OG E N ES I S

the arm of a young man

Abnormal fol l i c u l a r keratin ization of the u pper sectio n of
the h a i r fol l icle that may later res u lt in atro p h i c fo l l i c u l a r
sca rring.

PHYS I CAL EXAM I NAT I O N
Fol l i c u l a r

pl u gging

with

erythema

in

early

stages

( Figu re 33. 1 ) . Atro p h i c fol l i c u l a r sca r fo rmation with
assoc iated a lopecia in later stages .

D I FFERENTIAL D I AG N OS I S
Ke ratos is p i l a ris, keratosis pila ris ru b ra , seborrheic der­
matitis ( KPA F ) , atopic d e rmatitis ( KFS D ) , other etiologies
of sca rring a l o pecia ( KFS D ) , acne sca rri ng (AV), Rom bo
syn d rome (AV ) , a n d K I D syn d rome ( K FS D ) .

D E R M ATOPAT H O LOGY
D i lated fo l l ic l es with fo l l i c u l a r hyperkeratosis and i nfla m ­
m a t i o n i n e a r l y stages . Fol l i c u l a r fi brosis a n d atrophy i n
later stages .

CO U RS E
The cou rse i s c h ro n i c with n o sponta n eous reso l ution .
With t i m e , the e ryt h e m ato u s fo l l i c u l a r hyperkeratotic
pa p u les i nvol u te i nto d e p ressed atro p h i c fo l l i c u l a r sca rs
with a l opec i a .


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Color Atlas of Cosmetic Dermatology

MANAG E M ENT
There is n o com pletely effective treatment for KPA.
M u ltiple treatment options have been tried with only va ri­
a b le s uccess . Patients should be cou nseled that thera py
may not be effective.
•

Topical thera py may, at best, prod uce modest benefit.
- Lactic acid a n d a-hyd roxy acid lotions ( 1 0 %- 1 2 % )
a p plied twice d a i ly may i m p rove the text u ra l ro ugh­
ness. H owever, they may p rod uce i rritatio n .
- R eti n o i d s (taza rote n e , reti n-A) a p p l ied n i ghtly may
i m p rove text u r a l ro ugh ness. T h ey may prod uce i rri­
tati o n .
- Corticosteroids a p p l ied s pa ri ngly m a y show i m provement. R i s k of fac i a l atro ph y l i m its their use.

•

A

System i c thera py
- Other o ptions that have p rovided va ria ble su ccess
i n c l u d e o ra l reti noids a n d d a pso n e .
- They a re m ost h e l pfu l fo r the i nfla m m atory stage of
KPA, but provide m i n i m a l i m prove ment in the fol l ic u ­
l a r hyperkeratos is.
- They req u i re ca refu l mon itoring for potentia l side
effects.

•

Laser thera py
- P u lsed dye laser ( 59 5 n m , 7-m m spot, 7-1 0 J/cm 2 ,
D C D 40/20, p u lse d u ration of 1 . 5-3 ms) c a n be
effective in the treatment of the assoc iated e rythema
of KPAF but will not sign ifica ntly i m prove the text u ra l
rough n ess o f KPA ( Fig. 33 . 2A , B ) .
- Laser-assisted h a i r remova l with long- p u lsed n o n ­
Q-switc hed ru by l a s e r may be a n effective treatment
i n patients with KFS D .

P I T FALLS T O AVO I D
Pati ent expectations a re ge nera l ly very h i g h . They m ust
be cou nseled as to the c h ro n i c natu re of the cond ition
and m i n i m a l res ponse to ava i la ble thera pies.

B I B L I OG RAPHY
Baden H P, Byers H R . C l i n i c a l fi n d i ngs, c uta neous pathol­
ogy, and response to therapy i n 21 patients with keratosis
p i l a ris atro p h ica n s . Arch Dermatol. 1 994; 130(4):469475.
C h u i CT, B e rger TG , P rice VH, Za c h a ry CB. R eca lcitra nt
sca rring fol l ic u l a r d isord e rs treated by laser-assisted h a i r
re mova l : A prel i m i na ry report. Dermatol Surg. 1 999 ;
25( 1 ) : 34-3 7 .
C l a rk S M , M i l l s C M , La n iga n SW. Treatment o f keratosis
p i l a ris atro p h i c a n s with the p u lsed tunable dye laser. J

Cutan Laser Ther. 2000 ; 2 (3 ) : 1 5 1 - 1 56.

B

Figure 33.2 (A) Keratosis pilaris atrophicans. Patient is emotionally both­
ered by persistent erythema. (8) Marked lightening of erythema 2 years
following three pulsed dye laser treatments


Sect i o n 6: Va sc u l a r A l te rat i o n s

Ka u n e K M , Haas E, E m m e rt S, Schon M P, Z utt M .
Successfu l treatment of severe keratos is p i l a ris ru bra with
a 595- n m pu lsed dye laser. Dermatol Surg. 2009 ; 3 5 :
1 592- 1 595.
M a rq ue l i ng AL, G i l l ia m AE, P rend ivi l l e J, et al. Keratosis
p i l a ris ru b ra : A c o m m o n but u n d errecogn ized conditi o n .
Arch Dermatol. 2006; 142( 1 2 ) : 1 6 1 1 - 1 6 1 6 .
R i c h a rd

G,

H a rth W . Keratosis fol l ic u l a ris s p i n u losa

d ecalva n s . T he ra py with isotret i n o i n and etreti nate in the
i nfla m matory stage. Hautarzt. 1 993;44(8) : 529-534.

CHAPT E R 34

Po rt-wi n e Stains

Port-wine sta i n s ( PWS) a re low-flow ca p i l lary m a lforma­
tions. They represent the m ost common type of vasc u l a r
ma lformations. Any a rea o f t h e body can b e affected .
H owever, the head a n d neck a reas a re m ost co m mo n ly
affected .

EPI D E M I O LOGY
Incidence: 3 per 1 , 000 newborns
Age: prese nt at b i rt h i n the majo rity of patients ; rarely
a p pea r i n adolesce nce o r a d u lthood
Sex: no sex pred i l ection
Race: less common i n Asi a n s a n d African Americans
Associated syndromes: PWS can be a m a n ifestation of
severa l synd romes i n c l u d i n g Stu rge-We ber syn d rome,
K l i ppel-Tre n a u nay synd ro m e , P rote us syn d rome, and
pha komatos is pigmentovasc u la ris

P H YS I CAL EXA M I NAT I O N
PWS prese nts a t b i rth a s l ight p i n k , we l l-dema rcated
m a c u l a r lesions a n d patc hes usua l l y in a segmenta l d is­
tri butio n . They ca n tra n sform with age i nto hypertro p h i c
d a r k r e d a n d/or p u r p u ric pla q u es w i t h nod u l a rity. PWS
i nvolves the face m ost c o m m o n l y a l ong the trigem i n a l
n e rve d istri bution : ophtha l m i c b ra n c h V 1 ( u pper eye l i d
a n d forehea d ) , maxi l l a ry b ra n c h V2 ( u pper l i p , cheek,
lower eye l id ) , a n d m a n d i b u l a r b ra n c h V3 .

D I FFERENTIAL D I AG N OS I S
PWS exh i bits c h a racteristic c l i n i cal featu res a n d i s sel­
d o m m isd iagnosed . I t can be confused with the mac u l a r

stage o f h e m a ngioma at b i rth .

I

1 83


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Color Atlas of Cosmetic Dermatology

D E R M ATOPAT H O LOGY
M u ltiple d i lated t h i n -wa l led vesse ls in the pa p i l l a ry a n d
reti c u l a r d e r m i s .

A N C I LLARY TESTS
•

The pa rents s h o u l d be cou nseled rega rd i n g the possi­
b i l ity of Stu rge-We ber synd rome (SWS) i n lesions
l ocated i n a fac i a l Vl o r V2 dermatom a l d istri bution .
SWS is cha racterized by the prese nce of fac i a l PWS
with i psi latera l o c u l a r a n d lepto m e n i ngea l a n o m a l ies.
Ten to fifteen percent of pati ents with PWS i n the V l
d istr i b ution wi l l have SWS . Patients w i t h b i latera l PWS
h ave even a h igher risk of SWS . An ophthal mologic
exa m i nation to ru l e out gla ucoma a nd cata ract forma­
tion with conti n ued fo l lowu p is necessa ry for these

patients . A head c o m p uted tomogra phy ( CT) or mag-

A

netic reson a n ce i maging ( M R I ) s h o u l d be o bta i ned to
r u l e out b ra i n i nvolvement that could affect menta l
development a n d res u l t i n sei z u res.
•

PWS overlyi ng the s p i n e ca n be associated with s p i n a l
a n o m a l y s u c h as s p i n a l dysra p h i s m o r tethered s p i n a l
cord . N e u ro l ogic eva l uation a n d a p p ro priate i maging
stu d ies a re recom m e n d ed .

•

Large extremity PWS should ra ise the consideration of
Kl i ppel-Trenau nay syn d rome, cha racterized by capillary­
venous ma lformations or ca pil lary-lym phatic-venous mal­
formations with hypertrophy of the affected extrem ity. Leg
girth and length should be measu red and followed over
time.

COU RS E
PWS grows proporti o n a l l y with the patient a n d gra d ua l ly
t h i c kens a n d d a rkens i n color from p i n k to d a r k red to

B

deep p u rple. Eleven percent may d eve l o p n od u l a rity a n d

2 4 % may d eve l o p pyoge n i c gra n u lomas. PWS may b e
associated with hypertro phy o f u n derlying soft tissue a n d
bone,

pa rtic u l a rly

in

Stu rge-We ber

syn d rome

and

K l i ppel-Tre n a u nay syn d ro m e .

KEY CO N S U LTAT I V E QU EST I O N S
•

On set o f lesion

•

Assoc iated c l i nical fi n d i ngs

•

Is the c h i l d m eeti ng d eve l o pmenta l m i lestones?

•

Has the c h i l d had an eye exa m i nation?

•

Has the c h i l d had a head M R I or CT?

•

Past treatments a n d response

•

B l eed i ng

•

B l ebs

(B) Significant lightening of the PWS after a single POL treatment.

•

G rowth of PWS

(C) Complete resolution of the PWS after POL treatments

c

Figure 34. 1 (A) PWS on the right inner thigh of an infant girl.

Sect i o n 6: Va sc u l a r A l te rat i o n s

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1 85

MANAG E M ENT
PWS d e m o nstrates progressive vasc u l a r d i latation a n d
hypertrophy with age, t h u s m a k i ng treatment d u ri ng
ea rly i nfa ncy esse ntial for a bette r res ponse. Treatment
ca n be sta rted as ea rly as 2 weeks of age . Treatment p ro­
vides a red uction in the n u m be r of vessels a n d d oes n ot
c o m p l ete ly rem ove the enti re lesio n . T h e refore , the PWS
may exh i bit some d a rke n i n g a n d t h i c ke n i ng over t i m e
despite

i n terventio n .

G e n e ra l

a n esthesia

m ight

be

needed for treati ng la rge PWS i n c h i ld re n .
•

Laser treatm e n t ( F igs . 34. 1-34. 5 ) .
P u lsed dye laser ( P O L) rema i n s the gol d sta n d a rd for

the treatment of PWS . Effective P O L pa ra meters i n c l u d e
wavele ngths o f 5 8 5 t o 600 n m , flue nces o f 6 t o 1 5 J/c m 2 ,
p u l se d u rations of 0.45 or 1 . 5 ms with cryogen spray

A

cool i n g (CSC). Fou r to twe lve laser sessions with 4-to-8week i nterva ls a re u s u a l l y req u i red in order to ach ieve
sign ificant b la n c h i n g of the PWS . Lower fl uen ces a re i n itia l ly uti l i zed for PWS off the face a n d in d a rker s k i n
types . The use o f e s c concom ita ntly d u ri n g P O L treatment sign ificantly dec reases the pa i n associated with the
proced u re a n d the i n c i d ence of bl istering. esc protects
the epidermis a n d a l l ows for d e l ivery of h igher flu ences,
resulting in more effective b l a n c h i ng of the PWS . P O L
treatm ent is fo l l owed b y tem pora ry p u r p u ra that usua l ly
resolves in 7 to 14 days. Complete l ighte n i ng of PWS with
POL treatment is a c h i eved i n l ess than 20% of PWS .
Resista nce to

P O L treatment

is

more freq ue ntly

encou nte red in deeper and hypertro p h i c PWS . H e l pful
m a n e u ve rs to potentiate the efficacy of P O L i n c l u d e
i n c reasi n g t h e fl u e n ces with adeq uate c ryogen cool i n g to

p rotect the epidermis a n d i n c reas i n g the wavelength u p
to 600 n m to ta rget deeper vesse ls. A pi lot study demon­
strated that PWS that a re treated with to pica l imiquimod
once d a i ly for 1 month after P O L exposu re m a n ifest
su perior b l a n c h i ng res ponse over time as compared to
P O L a l o n e . Another re port i n vestigated the c o m b i ned use
of POL and a topica l a n giogenesis i n h i bitor, rapamycin,
using the in vivo rodent wi n d ow c ha m ber mode l . There
was no reformation a n d reperfusion of blood vessels after
treatment with P O L fol l owed by topical ra pamyc i n for
14 d ays, i n contrast to P O L a l o n e . With extreme ca ution
to avo i d sca rring and dyspigmentatio n , it is poss i b l e to
treat P O L-resista nt PWS and deeper or hypertro p h i c
a d u lt P W S su ccessfu l ly w i t h longer wavele ngth lasers that
a l low d eeper penetration i nto the skin such as l ongp u l sed a l exa n d rite (755 n m ) laser, long-pu lsed N d :YAG
( 1 , 064 n m ) laser, and d u a l 595- n m P O L a n d 1 ,064- n m
N d :YAG laser cou pled w i t h adeq uate coo l i ng. U s e o f t h e
N d :YAG laser can be treac h e rous as there is a narrow
thera peutic ra nge. R isk of sca r ca n be sign ificant.
•

Light treatment: i ntense pu lsed l ight ( I P L ) may be effec­
tive in treatment of PWS , i n c l u d i n g P O L- resista nt PWS .
A green-ye l l ow waveband a n d lowest ava i l a ble p u lse

B
Figure 34.2 (A) Extensive port-wine stain on the right face and forehead

of an infant male. (8) Significant resolution after multiple treatments
with pulsed dye laser

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Color Atlas of Cosmetic Dermatology

d u ration s h o u l d be used , with s k i n coo l i ng. A recent
ra ndom ized c l i n ical tria l com pa r i ng P O L a n d I P L side
by side revea led a better efficacy a n d h igher patient
preference after POL treatment. P h otodyna m ic thera py
may a lso prove to be an a lternative efficacious treat­
ment for PWS .
•

Other treatment modal ities for PWS that can be effec­
tive i n c l u d e tattooing a n d cosmetic m a keu p .

P I T FALLS TO AVO I D
•

Patients s h o u l d be cou nseled that PWS d isplay a va ri­
a b le response to treatment. M o re extens ive and th icker
lesions respond less wel l when com pa red to su perfi c i a l
lesions. Fac i a l PWS responds best. P W S treatment effi-

A

cacy decreases as one d escends from face to feet, with

the lower extre m ities d isplaying the least treatment
benefit.
•

M u lt i p l e treatment sessions may be req u i red . B r u i s i n g
is a necessa ry side effect t o o bta i n efficacious thera py.

•

Laser treatment may prod uce "footpri nti ng" or o n ly pa r­
tial i m p rovement.

•

Treatme nts should be ceased when the patient is satis­
fied with l ighte n i ng, o r when n o fu rther benefit has
been noted , that is, afte r two su bseq uent treatments.

B I B L I OG RAPHY
Alste r TS, Ta nzi EL. C o m b i ned 595- n m a n d 1 , 064- n m
laser i rrad iation o f rec a l c itra nt a n d hypertro p h i c port­
wine sta i n s in

c h i l d ren a n d a d u lts.

Dermatol Surg.

2009 ; 3 5 ( 5 ) : 8 1 3-8 1 5 .
C h a n g CJ , Hsiao Y C , M i h m M C J r, N elson J S . P i lot stu d y

B

Figure 34.3 (A) Extensive port-wine stain on the right neck of a young

female. (B) Marked resolution of the port-wine stain after multiple treatments with pulsed dye laser

exa m i n i ng the com b i ned u s e o f p u lsed d y e l a s e r a n d topical l m i q u i mod versus laser a l o n e for treatment of port
wine sta i n b i rt h m a rks. Lasers Surg Med. 2008;40(9 ) :
605-6 1 0 .
C h a pas A M , Eickhorst K, G e ron e m u s R G . Efficacy of
early treatment of fac i a l port w i n e sta i n s in newborns: A
review of 49 cases. Lasers Surg Med. 2007;39 ( 7 ) : 563568 .
C h i u C H , C h a n H H , H o WS , Ye u ng C K , N e lson J S .
P ros pective stu d y o f p u l sed d ye laser i n conj u nction with
c ryogen s p ray coo l i n g fo r treatment of port wine sta i ns i n
C h i n ese patients. Dermatol Surg. 2003;29(9):909-9 1 5 .
Discussion 9 1 5 .
Fa u rsc h o u A , Togsverd- B o K , Zachariae C , Haedersdal
M. P u lsed dye laser vs . i ntense p u lsed l ight for po rt-wine
sta i ns : A ra nd o m ized side-by-side tria l with b l i n ded
res ponse eva l uati o n . Br J Dermatol. 2009 ; 1 60(2) :359-

�.

A

Figure 34.4 (A) Port-wine stain on the lower mucosal and cutaneous lip.


Sect i o n 6: Va sc u l a r A l te rat i o n s

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1 87

Ho WS, Ying SY, C h a n PC, C h a n H H . Treatment of port
wine sta i n s with i ntense pu lsed l ight: A prospective study.

Dermatol Surg. 2004;30(6):887-890.
H u i keshoven M, Koste r P H , d e B orgie CA, Beek J F, va n
Gernert M J , va n d e r H o rst C M . Reda rken i n g of port-wine
sta i n s 1 0 years after p u l sed-dye-laser treatment. N Eng! J

Med 2007;356( 1 2 ) : 1 235- 1 240.
Li L, Kon o T, G roff WF, C h a n H H , Kitazawa Y, N oza ki M .
Com parison study of a long-pu lse p u lsed dye laser a n d a
long-pu lse p u lsed a lexa nd rite laser in the treatment of
port w i n e sta i ns . J Cosmet Laser Ther. 2008; 1 0( 1 ) :

12-15.
P h u ng T L , O ble D A , J ia W , B enja m i n L E , M i h m M C J r,
N elson J S . Can the wo u n d hea l i ng res ponse of h u ma n
s k i n b e mod u l ated afte r laser treatment a n d t h e effects of
exposu re exte nded? I m pl ications on the c o m b i ned use of
the p u l sed dye laser a n d a topical a ngioge nesis i n h i bitor

B

fo r treatment of port wine sta i n b i rth ma rks . Lasers Surg

Figure 34.4 (Continued) (B) Significant lightening of port-wine stain after

Med. 2008;40( 1 ) : 1-5.
Se l i m M M , Ke l l y K M , N e lson J S, We nd elsc hafe r-Cra b b G ,
Ke n n edy WR , Z e l i c kson B D. Confocal m i c roscopy stu d y

three treatments with a combination of pulsed dye laser to the cutaneous lip
and vermilion and long-pulsed 1 , 064-nm Nd: YAG laser to the inner
mucosa/ lip and vermillion

o f nerves a n d blood vessels i n u ntreated a n d treated
portwine sta i ns : Pre l i m i n a ry o bservati ons. Dermatol Surg.

2004;30:892-897.
Ya ng M , Ya roslavsky A , Fari n e l l i , e t a l .

Long-pu lsed

neodym i u m : Yttri u m -a l u m i n u m -ga rnet laser treatment
for port-wi ne sta i n s . J Am Acad Dermatol. 2005 ; 52(3):

480-490.

Figure 34.5 Hypopigmentation, which can be permanen t, after aggres­
sive treatment of a PWS in an A frican-American patient


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CHAPT E R 3 5

Pyoge nic G ra n ulo m a

Pyoge n i c gra n u l o m a ( PG ) c a n be rega rded a s a benign
vasc u l a r tu m o r o r a s a reactive vasc u l a r process a risi ng
at sites of prev i o u s tra u m a or i rritat i o n . PG is a lso k n own
as l o b u l a r ca p i l l a ry h e m a n g i o m a , gra n u l o m a tela ng­
iectatic u m , a n d gra n u lo m a gravi d a r u m when p rese nting
o n t h e gi ngiva of preg n a n t wo m e n . I t commonly occ u rs
i n a reas of tra u ma i n c l u d i n g the face a n d finge rs .

EPI D E M I O LOGY
Incidence: c o m m o n
Age: most common i n c h i l d ren a n d yo u ng a d u lts
Precipitating factors: m i nor tra u ma , pregna n cy, laser treat­
ment of port-wi ne sta ins, isotretinoin

Figure 35. 1 Classic hemorrhagic pyogenic granuloma

PATHOG E N E S I S
Reactive neovasc u l a rization suggested b y c o m m o n asso­
c iation with preexisting tra u m a o r i rritation a n d l i m ited
growth ca pac ity.

PHYS I CAL EXAM I NAT I O N
Red t o violaceous, d o me-sha ped , friable

pa p u l e or

nod u le , 0.5 to 1 . 5 e m i n size, with s m ooth surfa ce that
freq uently ulcerates ( Figs. 35. 1 , 3 5 . 2 and 3 5 . 3 ) .

D I F F E R E N T I A L D I AG N OS ES
N od u l a r a me l a n otic m e l a n o m a , glomus tumor, h e m a n ­
gioma , sq u a m o us c e l l carci noma ( S C C ) ( F ig. 3 5 . 4 ) ,
nod u la r basa l cel l carc i n o m a , wa rt, bac i l l a ry a ngiomato­
sis, Ka posi 's sa rco m a , and m etastatic cancer.

D E R M ATOPAT H O LOGY
Wel l -circ u mscri bed exo phytic l o b u l a r pro l i feration of ca p­
i l l a ries with flattened a n d someti mes e roded overlyi n g
epidermis w i t h pe r i p hera l epidermal "colla rettes . "

COU RS E
P G u s u a l l y grows ra p i d ly over the cou rse of weeks o r
months a n d then sta b i l izes. It b l eeds freq u e ntly with
m i nor tra u ma and ca n persist i n d efin itely if n ot treated .

Figure 35.2 Pyogenic granuloma on the palm of a pregnant woman,
bleeding frequently


Sect i o n 6: Va sc u l a r A l te rat i o n s

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1 89

MANAG E M ENT
•

Laser treatment
- Pu lsed dye laser (585--600 n m , 0.45- 1 . 5 ms, 7-10 m m ,
6-- 1 5 J/cm 2, O C O 20-40/20 with or without d iascopy) is
a safe and effective device for the treatment of small
lesions and for ped iatric patients. Seria l treatments are
usua l ly req uired . Treatment is wel l tolerated without
anesthesia. A recent report suggested shave excision
followed by immed iate pu lse dye laser ( P OLl for larger
lesions. POL has been also reported to be effective i n
gi ngival PG. Nd:YAG laser c a n also be effective.
- Carbon d ioxi d e is effective . Lesional flatte n i ng is the
c l i n ica l end point. l ntra l esional l i doca i n e 1% is neces­
sa ry prior to treatment. Postoperative ca re req u i res
twice d a i ly cleansing with soa p a n d water a n d a p p l i ­
cation o f a nt i b i otic oi ntment over a 2 t o 6 wee ks heal­
i n g t i m e . Sca r formation is l i kely. A low rec u rrence
rate is noted .

•

S u rgical treatment: a l l treatments may res u lt in sca r for­

Figure 35.3 Pyogenic granuloma overlying a dermal nevus

mati o n .

- Shave exc ision fol l owed b y electrod essication o f t h e
base is t h e proced u re most c o m m o n l y e m p loyed .
Recu rrence is common ( Figs . 3 5 . 5 a n d 3 5 . 6 )
- El l i ptica l exc ision c a n be pe rformed w i t h l o w rec u r­
rence but wi l l leave a sca r
- Ligation of the base
- C ryos u rgery
•

Alternative treatment options i n c l ud e
- l m iq u i m od 5 % c rea m h a s been recently reported to
be effective in ped iatric patients a n d in patients with
recu rrent PG
- l ntralesional i njection of a bsol ute etha nol
- Scleroth erapy with monoetha nola m i n e oleate
- To pica l a l itreti n o i n (9- cis-ret i n oic c i d ) ge l , a d rug that
is used for the treatment of Ka pos i 's sa rcoma

P I T FALLS TO AVO I D
•

Patients s h o u l d be awa re that rec u rre nce is common
after treatment.

•

Patie nts s h o u l d be i nformed that all treatments may
result i n sca rring.

•

Amela notic melanoma as wel l as SCC and other skin can­
cers can m i mic PG . A biopsy should be performed for
any suspicious lesions in the a ppropriate c l i nical setti ng.

B I B L I OG RAPHY
B o u rguignon

R,

Paq uet

P,

P i e ra rd - F ra n c h i mont

C,

P i e ra rd G E . Treatment o f pyogen ic gra n u lomas with t h e
N d-YAG laser. J Dermatolog Treat. 2006; 1 7(4) : 247-249 .

Figure 35.4 Pyogenic granuloma mimicking a squamous cell carcinoma

on the left lower mucosa/ lip of a patient with multiple nonmelanoma
skin cancers


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Fa l l a h H , Fisc h e r G , Zaga re l l a S. Pyoge n i c gra n u loma i n
c h i ld re n : Treatment with to pical i m i q u i m od . A ustralas J

Dermatol. 2007;48(4) : 2 1 7-220
Kha n d p u r S , Sharma VK. S u ccessfu l treatment of m u lti­
p l e gi ngiva l pyoge n i c gra n u lomas with p u lsed-dye laser.

Indian J Dermatol Venereal Lepra/. 2008; 74( 3 ) : 275-27 7 .
M a loney D M , S c h m idt J D , D u v i c M . A l itreti n o i n g e l to
treat pyoge n i c gra n u loma . J Am Acad Dermatol. 2002 ;
47( 6 ) : 969-970.
Mats u m oto K, N a ka n is h i H, Seike T, Koiz u m i Y, M i h a ra K,
Ku bo Y. Treatment of pyogen i c gra n u loma with a scleros­
ing agent. Dermatol Surg. 200 1 ;27(6) : 52 1 -523 .
R a u l i n C, G reve B , H a m mes S. The combi ned conti n u ­
ouswave( pu I sed carbon d ioxide laser for treatment o f pyo­
gen i c gra n u lo m a . Arch Dermatol. 2002 ; 138( 1 ) :33-3 7 .
S u d A R , Ta n ST.

Pyoge n i c gra n u loma c o m p l icating

p u lsed -dye laser thera py for c h e rry a ngioma . J Plast

Reconstr Aesthet Surg. 2010;63(8) : 1 364- 1368.

A

B

Figure 35.5 (A) Shaving a hemorrhagic and painful pyogenic granuloma
on the plantar foot with # 1 5 blade. The specimen was sent for histological
confirmation. (B) Electrodessication of the residual pyogenic granuloma


Sect i o n 6: Va sc u l a r A l te rat i o n s

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A

B

Figure 3 5 . 6 (A) Biopsy-proven pyogenic granuloma on the right chin of a
young female. (8) Shave excision of pyogenic granuloma with Derma
Blade (Personna Medical, Verona, VA)