Life History of Cutaneous Vascular Lesions

in Sneddon’s Syndrome
BERNHARD ZELGER, MD, NORBERT SEPP, Md,
KURT W. SCHMID, MD, HELMUT HINTNER, MD,
GEORG KLEIN, MD, AND PETER 0, FRITSCH, MD

Sneddon’s syndrome is a potentially fatal arterio-occlusive disorder segments, resulting in a seemingly larger pattern; occurs
characterized by generalized livedo racemosa and cerebrovascular on the trunk as well as the extremities; and persists OII
lesions. Skin biopsies often fail to yield diagnostic arterial lesions. warming of the skin. Despite these differences, no dis-
In the present series, affected vessels were found in skin biopsies
tinction is usually made in the English literature between
from 12 of 15 patients with Sneddon’s syndrome. Selection of the
these two subtypes, which are both summarized under
correct biopsy site (seemingly uninvolved skin at the center of a
the heading “livedo reticularis.” Ne\;ertheless, this dis-
livedo racemosa area), adequate biopsy size (1 to 2 cm), and serial
sections are essential for the detection of relevant vascular pathol- tinction is far from trivial, owing to their different
ogy. Only small to medium-sized arteries of the dermis-subcutis pathophysiologic basis and prognostic s@ificance. Both
boundary were found to be involved. Lesions follow a distinct types of livedo are caused’ by a slowed blood flow and,
course. The initial stage displays partial detachment of endothelial thus, lowered oxygen tension in the skin, which is lowest
cells, adhesion of mononuclear cells interspersed with fibrin (“en- at the peripheries of the cone-like tissue segments sup-
dothelitis”), a marked edema of the surrounding connective tissue plied by indiviciual central arteries. As a consequence,
with numerous dilated capillaries, and a predominantly lympho- the peripheries appear as dark livid rings around central
histiocytic infiltrate with polymorphonuclear leukocytes. In the early
areas of normal skin color. In livedo reticularis, the rc-
phase a sponge-like plug is formed from mononuclear cells, fibrin,
sulting netlike pattern is regular due to a generalized
and red blood cells, leading to partial to complete obstruction. A
perivascular inflammatory infiltrate, devoid of polymorphonuclear
reduction of the blood tlow, usually conseyuent to
leukocytes, is located around affected arteries; in the adventitia of functional disturbances (eg, vasoconstt-ic.tit,n in rhe
the occluded vessel, a very regular corona of dilated capillaries cold). In contrast, livedo racemosa results from irregular
appears that is continuous with more peripheral dilated and focal, persistent, and usually physical impairment of the
branching vessels. Organization of the occluding plug ensues in blood flow, such as arterial occlusion in atherosclerosis,
the intermediate stage by “subendothelial cell proliferation,” most vasculitides, or Snecidon’s syndrome.
likely due to immigrating smooth muscle cells. In the final stage, This anatomic consideration has important impli-
the occluding artery undergoes fibrosis, shrinkage, and atrophy. cations for the histopathologic diagnosis of Sneddon’~
HUM PATHOL 23:668-675. Copyright @ 1992 by W.B. Saunders
svncirome. To find the relevant vascular lesions, it is
Company
necessary to examine the central supplying artery;
Sneddon’s syndrome is an infrequent neurocuta- therefore, fairly large biopsy specimens must be taken
nexus disorder, first described by Sneddon in 1965,’ from the seemingly normal center of the tissue cone,
that is characterized by partial to complete occlusion of and even then serial sections are often necessaq. Biopsy
medium-sized arteries due to “intima proliferation” of specimens from the iiiuch more conspicuous violaceous
unknown or&in. It is characterized by generalized livedo circular segments ):ield no relevant information; this rna~
racemosa and a wide variety of neurologic symptoms explain whv biopsies in Sneddon’s syndrome are often
ranging from transitory ischemic attacks, sensory dys- referred to as “norlc.ontributo~“‘~“~” and why relativel,
functions, seizures, psychiatric disturbances, and intel- little is known about the characteristic vascular changes
lectual impairment to full-blown ischemic stroke. The of this disorder.
disorder is slowly progressive and often fatal due to In this stuciy, we report the histopathologic findings
neurologic complications. Involvement of other organs in 15 patients with Sneddon’s syndrome. The data gath-
may occur,’ but the disorder is most often asymptomatic. ered from this largest series permit delineation of a dis-
As a rule, laboratory values are within the normal range. tinct time sequence of the vascular processes that char-
“Livedo racemosa,” a term first coined by Ehrmann acterize this disorcier.
in 190’7.” is a violaceous netlike patterning of the skin
(Fig 1) similar to livedo reticularis, from which it differs MATERIALS AND METHODS
in several aspects: it consists of irregular, broken circular

668
 VASCULAR LESIONS IN SNEDDON’S SYNDROME (Zelger et al)

FIGURE 1. The skin appearance of livedo

rat :emosa i:patient no 3).

TABLE 1. Patient Data of Present Study

(“I \lKI hI’i”.( ‘I

I I t III

t XI1 t I\’
I\’
t III
Vi) Nl) t I\’
t Nl) Ul) I\’
I\’
L,
- z II
II
t III
+ t + III

t I\’
+ + i I\’
Nl) t I t II
+ NI) t III
I
c Nl) Nil \\ I.

Nl) ril) f u\ I.
+ Nil i \\.I.

669
 HUMAN PATHOLOGY Volume 23, No. 6 (June 1992)

FIGURE 2. Initial phase

(“endothelitis”). (a) Involved
artery (arrow) at the dermis-
subcutis (D. S) boundary.
Note perivasculor edema,
marked inflammatory infil-
trate, and dilated blood
vessels In the vicinity. (He-
matoxylin-eosin stain mag-
nification * 118.) (b) Close-
up of Fig 3a. Detachment
(arrows “1”) and rounding
up of endotheliol cells, at-
tachment of mononuclear
cells (arrows “2”). and fibrin
deposits (asterisk). (Hemo-
toxylin-eosin stain; mognifi-
cation ~‘1,254.) (c) Close-up
of Fig 3a. Presence of poly-
morphonucleor leukocytes
(arrows) within the mono-
nuclear perivascular infil-
trate. (Iiematoxylin-eosin
stain; magniftcation r:627.)

mato~~litI-eo?itI and otxx+tr. .fwo hundred wctiolrb of ;Itfwtrrl I?Ii/id Phnsr (Stccgr I). ‘l‘he earliest ohservcd httlgr
vessels werr studied alld ~illaly~eti with ;I ItIilliIneteI~ .scxle 1’01. is ;I Ioosetiitig and puti;il tlet~t~htiteilt of‘ itirlividtt;il en-
the smallest t~‘attsvcrs~ wsscl &ttteter without tunica dw~titia
dothelial cells (Ks); single or clusters of lytttphohistio-
and f01- the length of periplicral eloitgatrd capillm.ies (see Ix-
wtic c.ells (l.HCs), p;tiIiallv itttci-spetwd with filwin. are
low) iti the v~is~ul;Ir fdrsus of the upper 01. IIIidtlle dertttis.
attached lo these Ws, pi-efet-entially at their lutitirtal
aspwts (“ettdothelitis”) (Pig t’h). Thr tuttim tttctlia. itt-
RESULTS
Cludittg the lantitta elastica, is uiialtetxd at this stage.
Type and Location of Affected Vessels but thr xlvrittitia lavc7 and lfw stirrottiidiiig umiifxti\~e
Onfy sitdl to inctliuiti-sized arteries, cfwxterizecf tissue appear edettt:~tcms, with ati ittc~txxsccl ttttntbet- of
by a distinct elastica interna,
fatnina were involved; theit dilated capillaries (Fib r 2~) and a conspicuous predottt-
diameters ranged ft-cm 0.05 to 0.24 inii~ (mean range, ittatttly l.H(: infiltrate (Table 2) with I)oIYttto~Ptlotltt~l~~tt
0. I!? * 0.05 mm). Only xteties 21 the <lel-rttis-subctttis leukoc~ytcs (Fig 2~).
border were afl‘ected. Genules were consistently normal. f%~l~ Pha.w (Stccgt~ II).As (Irenext step. tlt~ Iuttwn
Time Sequence of the Arterial Lesions is partially or c~otttpltitel~ occludecl ly ;I thl-otnl~tts-like
plug of “stimulated” tmmot~ttcle3t~ dls with indistinct
‘4 cle;w time of lesions emerged
st~fueiicc fr-on1 the f;titi( c.ytoplasttis and large etichrottiatic-, t-otttttl to ov~tl,
histologic data (Figs L, through 5) as well as the quail- but itt ‘times it-regulai- 01’ kidttry-shapecl ttriclei wit 11 oc-
titative data (Table 2 md Fig 6) of the 18 arteries itt- casioml mitoses. ‘l‘hesc cells form ;I spotqe-liktx strw~ tlrc
\,estigaletl. that iItcorporates some fibrin and erythro~vtes in its itt-

670
 VASCULAR LESIONS IN SNEDDON’S SYNDROME (Zelger et al)

TABLE 2. Vascular Changes in Sneddon’s Syndrome

SLl@?

trrstiws (Fig Ai). I‘he I1lnic.a tnedia, including the elastica LatP Phmr (Stclgp II’). At the final stage. the oc-
itttertta, still appe;~rs ttcmttal at this stage (Fig 3d). The cluding plug is ahnost acellular, ovc4y fibrotic. and hv-
atl~entitia I;tyct. of‘ completely occluded arteries sh0it.s alitCed (Fig .%). With polarized light microscopy, Iii-.
;I ~c‘1.1’tq@ar “corona” of‘ ectatic capillaries (Fig 3d), refringent collagen fibers can be visuabed (Fig .5b). The
LV~IWY~Sonlv partially obstructed arteries have ttotw. In latttina elastica interna, the tuttica muscularis, and the
atltlitiott, ~~~c’t‘~~~tt~lic.;~l~tt~l~ oriented, tttarkedl~~ dilated, advetititial elastic fibers are ptwgt~essiwl~ changing, as
at~tl stt~clcht~d out vessels appear to branch out and ram- described above (Fig SC). In addition. the latnina elastica
if\ into the. more peripheral adverttitia rissur and the interna is I‘ocally degradated (Fig 51). occxsionally split-
Iowct. cktxrtis (Fig 31)). The uppet- dermis shows only an ting into t\vo layer-s (Fig 5e and f). .?\t-teries are either
incrmsed ttutnbet- of capillaries (Fig 3~) chat appeal cwtnpletel~ occluded (Fig :ia, h. arid c) (JI‘ recanalioxl
dottg;~tcd .III~ c.oiled. Neighboring artrries of rhe de]-- (Fig 51, c’. attd f). Occasionally, a (x)ntte( tion between
ink-wlxwl is lx~t-d~.t- appear dilated ancl engorged with thr ‘*cot.ona” of capillaries and the I-e~asc~tlarized htrnett
(XI\ Ihr0c‘ytt.s attd,;ot~ st~tuttt. suggesting stasis phenoni- kcomes Csible (Fig 5d). Vessel diatnetet~ (Table 2 and
c’ita. lit all sprc~itn~ts of this stage, er)-.throc.yte exrra- Fig 6) kc-rex~s, at~d the “corona” 01 $1+illaries and
\‘;lsates Uabk 2) \~rre foiintl in the vessel \valls atid,/ot~ 1,et‘l”tl’li~ttl;tt.l~ oriented \essrls in\~olutes ,qadrtally (Fig
iii their it~ttttcdiate Vicitiitv (also siderophag-es iii one 3). l,~tiiptiotiistio~~tic cell infilttxes arc al)senl.
c,xx). I’eri\xc~ular l,tiC ittfiltratcs (Table 2), devoid of‘ tiigut C’7 suttttttari~~s out- findings ~c~litmatica11~.
IICWIrophils, arc scc’tt around the occluded artcrics (Fig
311) x~tl. Athottg-It tnuc~h Icss c.ottspicuous. around the Relationship of Histopathologic Features to
pc7iphrral \-essrla. the Clinical Course
I~tr~rir~li~~/f~/%n.~~(Sltrgf~ 111). Later on, the occlusi\~e No cx)rrc.lation hctweett the duration of thtz disease
plug is higltily cellular, but the nuclei appwr regular and and the tiistop~tthologi~ stage of \~ascul;it~ lesions was
111011011101.~pt1011s. 1-01111t1 to oval, and p~c‘tiotic‘. Their c\‘- found. Both early and late lesions wre ol)serwd in pa-
t( ~pl;tstt~ is tttostly faint with distinct ceil bordtm (Fig 4a tients tvirlt sytttptottts of long as \vell ;t> short standing
at~d I)); less ollrtt 01 01~1~li~rlly, the cells appear to be (I‘al~le I ). In the two specimens that contained two in-
rtt~l~~dd~tl in ;I hht oti~ii~ttous basophilic matrix lvithour diviclual involved arteries. the ~asculat- chmges were of’
distinct cell ttttmhrat~~s (Fig 4c and d). These changes the same stag? in one instance (patient no. 6. early phase)
~~orr~spottd 11) “sut~rtidoth~lial cell l,rotif’~ratioii.” and of different stages in the other (patient tto. 1 1, initial
k:t-vrtttx)c-)~.~~and fibritt arc’ absent. The lantina elastica and early phases). In four patients in whont two biopsies
itiI&tta appeatwl partially collapsed and clutiipy in three Lvet-e pet-fi~tmed within 1 year, \ascrtlar changes wet-c>
01 limt. s~~r~c~itnrtts wifh ktstica stain. The Iavers of of the smte Stage in three instant es (bolft earl57 phases
st1too0t IHIIWIV c.ells it1 the tunica media decrease (Table in paticttI no. 6, both late phases in patirtits no. 2 and
21, whik* IItc. cslas(ic. fibers of the tuttiw media and ad- 3) and of different stages in one case (inttrtnediate and
\,c7ttitia ap~~2tt~ itrctx3s~d iii tiutiiher. bkcepl for one initial pliawi iii patient no. 12).
;tt.ttm witlt ottl\ par1ial ocx.lusion (Fig 4d). aH other ves-
‘rt.154tcnwcl twattali/ittg capillaries (Fig la. I,, and c‘; DISCUSSION
‘l,ilG 2) aiid Itad 2 pt~otiiit~etit “cotxmt” of c~apillaries
,~rtd I’t’t‘l’c’tt’li~rrlar 1)’c)tGwted \wsels (Table 2). In con-
trast to pxrtial oc~c~lusioti, which is characterized by a
\ittgk, IMIWN. lat,ge (.rcscettt-like htmen (Fig 4d). rc- First, it ia most itnportant to etrtphasi:~c~ the ttecessity
c~ati;tli/ed \ t3scIs Itxr cme or several sntall, routtd lutnina of‘ selecting the appropriate hiopsv siIt.. c)f tal\ing suf-
(l:ig k). l’~t~i\;t4cul;tt~ I .t-I(i infiltrates were ahwnt in ;iII ficieti~l!~ G/et1 speciniens, and ot‘ wading trial sections
ht11 0tw oft k ;tfktcvl at-tel-ies (which was rathct- sparse), in r~txlrt~ IO detect the r-elevxit v;isctilar Irsioris. Whilr
~3Itik the titor~ aupet+icktl papillary pksus still exhibited the tiisIo~~;ttliologi~ resu)t5 of‘ others arc tttireliable and
;I 1’~ pc’ri\,asc III;II- I.H(:s. t BIteI “tir~tic~otitrit~iitor~,” ati HOY 4ttc‘(.(:\4 rate in ottt
671
HUMAN PATHOLOGY Volume 23. No. 6 (June 1992)

FIGURE 3. Early phase. (a)

Complete arterial occlusion
by a sponge-like plug of
mononuclear cells, contain-
ing fibrin (arrows) and er-
ythrocvtes in its interstices.
(Hemotoxylin-eosin stain;
magnificatron x384.) (b)
Vascular phenomena in the
vicinity of a totally occluded
artery: “corona” of dilated
capillaries in the adventitia
(arrows) contiguous with di-
lated and branching vessels
more perrpherolly. Note the
absence of polymorpho-
nuclear leukocytes in the
perivascular infiltrate. (He-
matoxylin-eosin stain; mag-
nification ~240.) (c) Upper
dermis with markedly dilated
and elongated capillary.
(Hematoxylin-eosin stain;
magnitication x 110.) (d)
Complete arterial occlusion
by a plug of mononuclear
cells: unaltered lamina elas-
tico interno and conspicuous
“corona” of ectatic capil-
laries. (Elastica stain; mag-
nification ~216.)

FIGURE 4. Intermediate
phase (“subendothelial cell
proliferation”) Organization
of the occluding plug is evi-
dent by its solid, more cel-
lular character. (a) Cells with
regular, round to oval, pyc-
notic nuclei (arrows) and
nearly no collagen fibers.
(Hematoxylrn-eosin stain;
x336.) (b) Close-up of 5a.
Note the monomorphous
cell character with faint cy-
toplasm and distinct cell
borders, and collapsed and
focally. slightly degradated
lamina elastica interna (ar-
rows). Elastica stain; magni-
fication X540.) (c) The mon-
omorphous cells exhibit no
distinct cell membranes and
appear to be embedded in
a ftbromucinous basophilic
matrix. Note two capillary
lumina within the plug (ar-
rows) and the prominent
“corona” of dilated adven-
titial capillaries indicating
recanalization. (Hematoxy-
lin-eosin starn: magnification
X180.) (d) Artery with in-
complete occlusion. Note
absence of dilated adven-
titial capillaries (Hematoxy-
lin-eosin stain; magnification
r 180.)

672
 VASCULAR LESIONS IN SNEDDON’S SYNDROME (Zelger et al)

FIGURE 5. Late phase (a) Poorly cellular, hyallnized, and fibrotic occluding plug (Hematoxylin-eosin stain, magnificotlon - 244.)
(b) Polarized light microscopy of the same vessel: birefringent intralumlnal collagen fibers (Hematoxylin- eosin stain, polarized light;
magnification q.244.) (c) Clumpy and distorted lamina elastica interna (asterisk) in a fibrotic, shrunken, late-stage artery. (Elastica
stain: magnification x408.) (d) Marked focal degradation of lamina elastica Interna (arrows) and atrophic turlica media. Note
apparent connectlon between involuting “corona” of capillaries and revasculanzed lumen. (Elastica stain; magnification 1 164 )
(e) Recanolized artery with atrophic tunica media and involuted “corona” of capillaries Note ftbrosis of plug (asterisk) and focal
remnants of the original lamina elastica interna (arrows). (Hematoxylin-eosin stain, magnification ’ 339.) (f:l Elcstica stain of the
same vessel reveals splitting of lamina elastica interna into two layers. (Elastica stain; magnification . 339 )
 HUMAN PATHOLOGY Volume 23, No. 6 (June 1992)

ized by detachment of individual ECs and adhesion of

lymphocytes. This hitherto unknown initial stage of
Sneddon’s syndrome is reminiscent of the earliest
changes observed in acute vascular renal allograft
rejection”) and accordingly has been labeled “endoth-
elitis” by us. This finding has several important impli-
cations: obviously, the primary target tissue in this dis-
order appears to be the endothelium, and the
“subendothelial proliferation,” which dominates later
stages, is likely to be a secondary phenomenon. Fur-
thermore, lymphocyte adhesion points to underlying
immunologic mechanisms of unclear origin and makes
it difficult to maintain the view that Sneddon’s syndrome
is a fundamentally noninflammatory disorder. Endo-
thelial cytotoxic activity of serum from Sneddon’s pa-
tients in cell cultures’” may be a clue to explain the
pathogenetic events in these very early stages of vascular
lesions. It remains unclear, however, why only ECs of
small to medium-sized arteries are affected. Presumably,
a specific pattern of adhesion molecules on the lym-
FIGURE 6. The vessel diameter (on y-axis in micrometers) in phocyte and endothelial surfaces is required to initiate
stage IV vessels is significantly smaller (P = .Ol) than in stages I this process.
to Ill (Mann Whitney U). The initial stage of “endothelitis” is likely to he
short lived (which could explain why it escaped detection
scribed above. This is consistent with the views that
for many decades); obviously, more lymphocytes and
Sneddon’s syndrome is (1) a noninflammatory disorder
monocytes are soon attracted to the early lesion, re-
characterized by “subendothelial proliferation” of im-
sulting in a sponge-like plug that traps erythrocytes and
migrating smooth muscle cells or myofibroblasts,” (2)
a “segmental hyalinizing vasculitis,“” (3) an “endarter- fibrin in its interstices and bears superficial resemblance
itis obliterans” described as “obstructive and obliterative to a thrombus (from which it can be distinguished by
changes secondary to intimal endothelial proliferation the relative paucity of red blood cells and fibrin in re-
within a fibromucinous matrix,““’ or (4) “a vessel with lation to prominent “stimulated” inflammatory cells
thickened wall, occluding thrombus, and no inflamma- within). This plug leads to partial or total occlusion of
tion.“15 Other researchers merely mention “narrowing the vascular lumen, which may account for the stasis
of the lumen,” “ hypertrophy of tunica media or arterial phenomena of neighboring arteries and erythrocyte ex-
walls, “2.1’i-1yor “inti*na p,_oliferation,“?n~~l travasates. In the case of complete obstruction, a num-
Pathologic alterations of the artery set out with a ber of vascular phenomena occur around the affected
very subtle disturbance of the endothelium, character- vessel: a very regular “corona” of dilated capillaries in

FIGURE 7. Life history of vascular lesions in Sneddon’s syndrome. (I) lnifial phase: “endothelitis”- detachment of ECs (arrows).
luminal attachment of LCHs (o), deposition of fibrin (w), perivascular edema with ectatic capillaries (&-y), and a predominantly
lymphohistiocytic infiltrate with polymorphonuclear leukocytes (0). Smooth muscle cells (0) of tunica media. (II) Early phase:
the lumen is occluded by a thrombuslike plug of ‘stimulated” mononuclear cells (0). erythrocytes @), and fibrin. Note the
“corona’ of ectatic capillaries in the adventitia layer and more peripheral, perpendicularly oriented branching vessels. The
perivascular lymphohistiocytic infiltrate is still marked. Polymorphonuclear leukocytes are absent. Note the focal erythrocyte
extravasates. (Ill) Intermediate phase: “subendothelial cell proliferation”-the artery is occluded by a cellular (0). only focally
fibrotic (e) plug, focal recanalization, collapsed membrana elastica interna, prominent ‘corona” in contrast to less
conspicuous perpendicularly oriented vessels, and sparse perivascular lymphohistiocytic infiltrate. (IV) Late phase: complete
occlusion (a) or recanalization (b). The artery is shrunken, the occluding plug is nearly acellular and fibrotic, the membrana
elastica interna is collapsed and clumpy (vvr), the tunica media is atrophic, the “corona” vessels and perpendicularly oriented
vascular plexus are almost completely involuted, and the perivascular inflammatory infiltrate is absent.

674
 VASCULAR LESIONS IN SNEDDON’S SYNDROME (Zelger et al)

the adventitia layer that are obviously contiguous to a turbance of blood viscosity and of blood flow. Br J Dermatol93:519-
system of equally dilated, long, and branched vessels 529,1975
5. Rump1 E, Rump1 H: Recurrent transient global amnesia in a
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reminiscent of vasa vasorum which, however, are only drome). J Neural 221:127-131, 1979
found in vessels larger than 1 mm in diameter.” These 6. Lubach D, Stamm T: Neurologische Veaderungen bei Livedo
vessels may be interpreted as a compensatory circuit. racemosa generalisata (Ehrmann)-Kasuistik und Literaturiibersicht.
Hautarzt 32:245-248, 1981
As an alternative explanation, they may be newly formed 7. Stevens WP, Ferguson LT: Livedo reticularis and cerebrovas-
by angiogenic factors released in the course of the dis- cular disease. Postgrad Med J 58:70-73, 1982
ease process (platelet-derived growth factor and basic 8. Rebello M, Val JV, Carijo F, et al: Livedo reticularis and ce-
fibroblast growth factor). 25-27These vascular networks rebrovascular lesions (Sneddon’s syndrome)-Clinical, radiological and
persist throughout the life cycle of the lesions, are most pathological features in eight cases. Brain 106:965-979, 1983
9. Rump1 E. Neuhofer J, Pallua A, et al: Cerebrovascular lesions
prominent in the intermediate stage, and gradually in- and livedo reticularis (Sneddon’s syndrome)-A progressive cerebro-
volute later on, roughly parallel to the recanalization of vascular disorder? J Neurol 23 1:324-330,1985
the occluding plug. 10. Comes A, Perroud AM, Mathieu A, et al: Livedo reticularis
Reorganization of the occluding plug is accom- et accidents vasculaires cerebraux. Ann Dermatol Venereol 113: 137-
141. 1986
plished by stimulation, immigration, and subendothelial
11. Scott IA, Boyle RS: Sneddon’s syndrome. Aust N 2 J Med
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in atherosclerosis and other instances of vascular dam- with cerebrovascular lesions (Sneddon’s syndrome): An occlusive ar-
age. ‘a This process is likely to be triggered by platelet- teriolopathy due to proliferation of medial smooth muscle cells. Br J
Dermatol 112:703-708, 1985
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resulting in collagen synthesis, vessel shrinkage, and in- and neurological lesions. Br J Dermatol 106:71 l-712, 1982
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volution of the occluded vessel; elastic tissue changes
J Am Acad Dermatol 16: 1084- 1087, 1987
most likely are due to this reparative process. At the 16. Trzebicki J, Buksowicz C: Zerebrale Angiographie bei einer
same time, perivascular inflammation gradually de- Livedo racemosa. Fortschr Geb Rontgenstr Nuklearmed Erganzung-
creases and finally disappears. A comparison of our hand 3:704-706, 1969
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niastej skory i Lmian moLgowych. Neurol Neurochir Pol 20:587-590,
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19. De Reus R, De Reuck J, Vermander F, et al: Livedo racemosa
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HH (eds): Fortschritte der Praktischen Dermatologie und Venerologie,
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