LUPUS ERYTHEMATOSTJS AND POLYMORPHOUSLIGHT ERUPTIONS

AN EXPERIMENTAL STUDY ON THEIR POSSIBLE RELATIONSHIP*

MILTON M. CAHN, M.D., EDWIN J. LEVY, M.D., BERTRAM SHAFFER, M.D.AND HERMAN BEERMAN, M.D.

The term chronic polymorphous light eruption, introduced by Hausmann andHaxthausen in 1929 (1), describes a "prurigo-like" as well as an eczemtous erup-tion, both of which are induced by exposure to sunlight. Other names for theminclude dermatopathia photogenica, erythema perstans solare, and prurigo aesti-valis (2). This paper, however, deals only with the prurigo-like eruption whichHutchinson (3) called "Summer Prurigo". Summer prurigo is characterized byrecurrent eruptions on those portions of the body exposed to sunlight. There maybe prodromal symptoms of burning or itching. Erythema and some degree ofurticaria-like swelling soon develop. The early lesions may fade but are usuallyreplaced by more or less persistent, discrete, inflammatory papules or plaqueswhich may become confluent resulting in diffuse involvement of the exposedskin. This condition commonly begins in late spring or early summer and usuallyterminates spontaneously in the fall. Remission often follows the use of sun-pro-tective measures (2, 4, 5). Lamb and his associates have recently elaborated onsome of the details of this affection (4).

During the month of June 1952 seventeen patients were observed in the out-patient department of the Skin and Cancer Hospital of Philadelphia, in whomthe differential diagnosis between subacute lupus erythematosus and the prurigoaestivalis type of polymorphic light eruption could not be made with certaintyeither by clinical or histologic examination. Accordingly, studies were institutedto determine whether there was any relationship between these two processes.

The results of our investigations lead us to suggest that lupus erythematosusis not a clear-cut concept, and certain reactions to light may at times representstages in transition from subclinical (or latent) to manifest clinical systemiclupus erythematosus. Both polymorphous light eruptions and lupus erythemato-sus may represent cutaneous expressions of a vasculo-allergic response to light (6).Exposures to sunlight in individuals who react in the abnormal fashion discussedin this paper, may finally precipitate the process called systemic lupus erythema-tosus.

* From the Skin and Cancer Hospital of Philadelphia (Bertram Shaffer, M.D., MedicalDirector), and the University of Pennsylvania, Departments of Dermatology, School ofMedicine (Donald M. Pillsbury, M.D., Director) and Graduate School of Medicine (HermanBeerman, M.D., Chairman).

This study supported in part by a grant from the Smith Kline and French Laborato-ries, Philadelphia.

Presented at the Fourteenth Annual Meeting of The Society for Investigative Derma-tology, Inc., New York, N. Y., May 30, 1953.

375

376 TEE JOURNAL OF INVESTIGATIVE DERMATOLOGY

MATERIAL

Our observations were made on 17 patients whose present or past history re-vealed a definite relationship between exposure to sunlight and the eruption.None of the patients had photosensitization by substances coming in contactwith the skin (6), or sensitization of the skin to light by medicarnents either exog-enous or endogenous (10). None of them had virus infections at the time of lightexposure (7).

Our patients were divided into three general groups. Group I: 9 patients witheruptions on the sites of exposure to sunlight. Morphologically their lesions wereidentical with those of prurigo aestivalis. The lesions cleared rapidly with sunprotective measures. There was no clinical or laboratory evidence of systemicabnormalities. Group II: 4 patients with recurrent, seasonal, transient eruptionsfor a period of several years, in the areas exposed to sunlight. These eruptionsalso cleared, but very slowly, after the use of sun protective measures. In addi-tion, these patients showed some abnormal laboratory findings, including aslightly elevated sedimentation rate, moderate anemia, and leukopenia, and atendency toward a reversal of the albumin/globulin ratio. Group III: 4 patientswith lesions of lupus erythematosus on the portions of the body exposed to sun-light. The lesions were chronic and persistent and some healed with atrophy.These patients also gave a past history of recurrent, transient eruptions for manyyears on the parts exposed to sunlight. There were certain abnormal laboratoryfindings. These, as well as the details of the clinical material are summarized inTables 1, 2, 3, and 4.

METHOD

Two and a half (2.5) centimeter square areas on the mid-portion of the backof each of the seventeen patients received exposures from the following lightsources:

(1) Ultraviolet light rays generated by a hot quartz, air cooled, mercury vaporburner at 75 centimeters from the test site for 10, 20, 30, 40, 60, and 120 seconds.

(2) Ultraviolet light rays generated by a cold quartz mercury vapor burner at20 centimeters from the test site for 10, 20, 30, 40, 60 and 120 seconds.

(3) Infra-red rays generated by a 250 watt Mazda bulb at 75 centimeters fromthe test site for 15 minutes.

(4) Wood's light, (generated by passing ultra-violet light rays through Wood'sifiter) at 10 centimeters for 15 minutes.

Five (5) centimeter square areas on the midportion of the back and "V" ofthe neck of each of 13 patients (those in groups I and II) received 5 minute ex-posures of ultraviolet light rays generated by a hot quartz mercury vaporburner at 75 centimeters from the test site. Exposures were repeated at weeklyintervals for 5 to 10 weeks to the same site.

Five (5.0) centimeter square areas on the midportion of the back and "V" ofthe neck of each of 9 patients (groups I and II) received a single 12 minute ex-posure of ultraviolet light rays generated by a hot quartz mercury vapor burner,75 centimeters from the test site.

TA

BL

E 1

Clin

ical

data

on

seve

ntee

n pat

ient

s with

pol

ymor

phou

s lig

ht e

rupt

ions

CA

SE

A

GE

1 42

E

. M.

SEX

fe-

mal

e

CO

MPL

EX

ION

light

ly p

ig-

men

ted;

m

any

frec

kles

RA

R

CO

LO

R

red

EY

E

CO

LO

R

blue

occu

p i

AT

ON

hous

ewif

e

USU

AL

RE

AC

TIO

N

TO

SU

NU

GH

T

sunb

urns

read

ily

and

tans

easi

ly,

but n

ot ex

ces-

si

vely

or p

atho

- lo

gica

lly

ON

SET

TIM

E

0? Y

EA

R

June

, pas

t 3

year

s

IMM

ED

IAT

E

RE

AC

TIO

N

eryt

hem

a an

d w

elts

in 2

hour

s af

ter e

xpos

ure;

di

sapp

ears

in 6

hour

s

ZY

ED

RE

AO

N

D

repl

aced

by

papu

les

in 2

4 ho

urs;

ery

- th

ema

rem

ains

BE

SUL

T 0?

USE

OF

SUN

PRO

TE

CT

1VE

S

prom

pt cl

eari

ng

in 5 d

ays;

rem

ains

cl

ear

LO

CA

TIO

N

OF

ER

UPT

ION

expo

sed

part

s,

face

, "V

" of

nec

k,

arm

s and

legs

DIA

GN

OSI

S AN

D

CO

MM

EN

TS

poly

mor

phou

s lig

ht

erup

tion;

pr

urig

o ae

stiv

ali

type

with

ur

ticar

ial

com

po-

nent

2 32

C

. G

. fe

mal

e lig

htly

pig

- m

ente

d;

man

y fr

eckl

es

blon

d gr

een

hous

ewif

e su

nbur

ns re

adily

an

d ta

ns e

asily

; no

t exc

essi

vely

or

path

olog

ical

ly

June

, pea

t 3

year

s er

ythe

ma

and

wel

t-lik

e les

ions

in

2 ho

urs,

last

- in

g 8

hour

s

diss

emin

ated

cr

y-

them

ato-

papu

lar

erup

tion i

n 24

hou

rs

prom

pt c

lear

ing

(5 d

ays)

, re

mai

ns

clea

r

expo

sed

part

s,

face

, "V

" of

nec

k,

arm

s and

legs

poly

mor

phou

s lig

ht

erup

tion;

pr

urig

o ae

stiv

alis

type

with

ur

ticar

ial c

ompo

nent

3 44

H

. W.

mal

e lig

htly

pig

- m

ente

d lig

ht

brow

n bl

ue

guar

d ou

t-

door

s au

nbur

ns n

or-

maf

ly; t

ans

read

- ily

and

nor

mal

ly

June

, pas

t 7

year

s in

tens

e cry

- th

ema

in 6

hour

s er

ythe

mat

o-pa

pula

r er

uptio

n in

24 h

ours

; m

axim

um in

48

hour

s

clea

rs in

5 da

ys

and

rem

ains

clea

r ex

pose

d pa

rts,

fa

ce, "

V"

of n

eck,

ar

ms a

nd le

gs

poly

mor

phou

s lig

ht

erup

tion;

pa

pula

r ty

pe

4 29

V

. S.

m

ale

light

ly p

ig-

men

ted

brow

n bl

ue

driv

er o

ut-

door

s su

nbur

ns n

or-

mal

ly; t

ans r

ead-

ily

and

nor

mal

ly

June

, pas

t 3

year

s in

tens

e cry

- th

ema

in 6

hour

s

papu

lar e

rupt

ion

in

18-2

4 ho

urs

clea

rs i

n 5-

10

days

; rem

ains

cl

ear

expo

sed

part

s,

face

, "V

" of

nec

k,

arm

s, a

nd le

gs

poly

mor

phou

s lig

ht

erup

tion;

pa

pula

r ty

pe

5 31

E

. MoM

.

mal

e lig

htly

pig

- m

ente

d br

own

blue

dr

iver

out

- do

ors

sunb

urns

nor

- m

ally

; tan

s rea

d-

ily an

d no

rmal

ly

June

, pas

t 2

year

s er

ythe

ma

in 24

ho

urs;

max

i-

mum

in 48

hou

rs

papu

les i

n 96

hou

rs

clea

rs in

7-1

4 da

ys; r

emai

ns

clea

r

limite

d to

left

el-

bo

w, e

xpos

ed t

o su

nlig

ht w

hile

dr

ivin

g

poly

mor

phou

s lig

ht

erup

tion;

lo

caliz

ed

papu

lar

type

6 32

C

. M.

mal

e m

oder

atel

y pi

gmen

ted

brow

n gr

ey

acet

ylen

e to

rch

wel

der

take

s lon

g tim

e to

sun

burn

; tan

s w

ell

Aug

ust 1

951,

July

195

2 er

ythe

ma

in 24

ho

urs

papu

les i

n 48

hou

rs;

beco

me

coal

esce

nt

clea

rs i

n 7-

14

days

; rem

ains

cl

ear

expo

sed

part

s,

face

, "V

" of

nec

k,

arm

s an

d le

gs

poly

mor

phou

s lig

ht

erup

tion;

pa

pula

r an

d pl

aque

-lik

e

7 41

R

. P.

8 40

S.

S.

mal

e

mal

e

mod

erat

ely

pigm

ente

d;

man

y fr

eckl

es

mod

erat

ely

pigm

ente

d

brow

n

brow

n

brow

n

brow

n

mec

hani

c

sale

swor

k ou

tdoo

rs

sunb

urns

with

dif

- fi

culty

; tans

dee

ply

and

rem

ains

pig

- m

ente

d

tans

eas

ily; d

oes

not b

urn

June

, pas

t 4

year

s

Apr

il 19

52,

3 tim

es s

um-

eryt

hem

a in

2 ho

urs

—

papu

les i

n 6-

24

hour

s

eryt

bem

ato-

papu

lar

and

disc

rete

disc

oid

clea

rs i

n 5-

14

days

; rem

ains

cl

ear

clea

rs in

7-1

5 da

ys;

rem

ains

clea

r Un-

expo

sed

part

s,

face

, "V

" of

nec

k,

arm

s an

d le

gs

expo

sed

part

s fa

ce,

"V"

of n

eck,

poly

mor

phou

s lig

ht

erup

tion;

pa

pula

r an

d pl

aque

-lik

e

poly

mor

plio

us

light

er

uptio

n; p

apul

ar

I

mci

' 195

2 an

d co

nflu

ent 1

0-

sion

s til

expo

sed

to

sunl

ight

ar

ms

and

legs

an

d pl

aque

-lik

e

TA

BL

E 1

—C

ontin

ued

CA

SE

AG

E

9 34

A

. T

.

SEX

mal

e

CO

MPL

EX

ION

dark

ly p

ig-

men

ted

RA

IR

CO

LO

I

brow

n

EY

E

CO

LO

R

brow

n

OC

CU

PAT

ION

pain

ter

outd

oors

USU

AL

RE

AC

TIO

N

To

SWqI

,IG

RT

tanS

eas

ily; d

oes

not b

urn

ON

SET

TIM

E

OP

YE

AR

June

, eac

h ye

ar si

nce

1945

IMM

ED

IAT

E

RE

AC

TIO

N

eryt

hem

a in

4-

24 h

ours

DE

LA

YE

D RE

AC

TIO

N

eryt

hem

ato-

papu

lar

and

disc

rete

dis

coid

an

d co

nflu

ent

le-

8iO

ns

RE

SUL

T O

P U

SE O

P SU

NPR

OT

EC

TIV

ES

clea

rs i

n 7-

15

days

; rem

ains

cl

ear u

ntil

ox-

pose

d to

sun

light

LO

CA

TIO

N O

P E

RU

PTIO

N

expo

sed

part

s fa

ce, "

V"

of n

eck,

ar

ms

and

legs

DIA

GN

OSI

S AN

D

CO

MM

EN

TS

poly

mor

phou

s lig

ht

erup

tion;

pa

pula

r an

d pl

aque

-lik

e typ

e

10

33

R. R

. fe

mal

e lig

htly

pig

- m

ente

d —

—

ho

usew

ife

—

June

194

3 an

d ea

ch

sum

mer

; la

st ti

me

June

52

prio

r to

1952

, er

ythe

ma

in 24

ho

urs;

in 1

952,

no

imm

edia

te

reac

tion

befo

re 1

952,

tran

- si

ent

papu

les i

n 24

hour

s; In

195

2, pa

p-

ules

per

sist

and

dis-

co

ld le

sion

s in

48

hour

s

clea

red i

n 5

days

; no

w le

sion

s pe

r-

slat

for

1 m

onth

face

onl

y "p

ruri

go"

type

of

po

lym

orph

ous

light

er

uptio

n or

suba

cute

lu

pus

eryt

bem

atos

us

28

fem

ale

dark

ly p

ig-

blac

k br

own

sten

og-

norm

al su

nbur

n ea

ch A

pril

eryt

hem

a of

be

fore

195

2, t

ran-

be

fore

195

2,

face

onl

y "p

ruri

go"

type

of

m

ente

d ra

pher

re

actio

n; ta

ns

and

June

fa

ce in

4—24

si

ent p

apul

es in

24

clea

red

prom

ptly

; po

lym

orph

ous

light

no

rmal

ly

sinc

e 19

50

hour

s ho

urs;

now

papu

les

now

lesi

ons

"com

e er

uptio

n or

suba

cute

in

48

hour

s and

an

d go

" lu

pus

eryt

liem

atos

us

cont

inue

to ap

pear

fo

r 2 w

eeks

12

34

D. S

. fe

mal

e da

rkly

pig

- m

ente

d bl

ack

brow

n ho

usew

ife

norm

al su

nbur

n re

actio

n; t

ans n

or-

mal

ly

atta

cks

each

su

mm

er; 1

st

one

Febr

u-

ary

1947

in

Flor

ida

inte

nse c

ry-

them

a in

4—24

hour

s

tran

sien

t pap

ules

pr

ior t

o 19

52; n

ow

papu

les i

n 48

hou

rs,

cont

inue

to ap

pear

, di

sapp

ear s

low

ly

clea

red

prom

ptly

be

fore

195

2; n

ow

tend

to p

ersi

st

and

go a

way

sl

owly

expo

sed

part

s fa

ce, "

V"

of n

eck,

an

d ar

ms

"pru

rigo

" ty

pe

of

poly

mor

phou

s lig

ht

erup

tion

or su

bacu

te

lupu

s er

ythe

mat

o-

sus;

his

tory

of

rheu

- m

atic

fev

er

(?)

in

June

195

1

13

42

G. C

. fe

mal

e lig

htly

pig

- m

ente

d br

own

blue

ho

usew

ife

norm

al su

nbur

n re

actio

n; ta

ns

norm

ally

firs

t atta

ck

in 19

33

(Jun

e); r

e-

curs

each

ye

ar in

Ju

ne

"wel

ts"

and

blot

ches

in a

fe

w h

ours

in 24

—48

hou

rs, e

ry-

them

atou

s, p

apu-

la

r, d

iscr

ete a

nd

conf

luen

t eru

ptio

n

clea

rs p

rom

ptly

in

7-1

0 da

ys

expo

sed

part

s fa

ce, "V

" of

nec

k an

d ar

ms

"pru

rigo

" ty

pe

of

poly

mor

phou

s lig

ht

erup

tion

or su

bacu

te

lupu

s er

ythe

mat

o-

sus;

hi

stor

y of

by-

pe

rten

sion

si

nce

June

1949

14

17

B. S

. fe

mal

e lig

htly

pig

- m

ante

d br

own

gree

n al

e k

norm

al su

nbur

n re

actio

n; t

ans n

or-

mal

ly

each

June

si

nce

1949

; pr

esen

t er

uptio

n in

N

ov. 1

952

eryt

hem

a in

2

hour

s; m

axim

um

in 2

4 ho

urs

past

yea

rs er

ythe

m-

atou

s pla

ques

in 6—

24 h

ours

; at p

res-

en

t, pa

pule

s con

- tin

ue to

form

form

erly

cle

ared

qu

ickl

y; n

ow d

o no

t cle

ar a

nd a

re

spre

adin

g

form

erly

only

ex-

po

sed

part

s; n

ow

on b

oth

expo

sed

and

unex

pose

d pa

rts

of th

e bo

dy

suba

cute

lupu

s er

y-

them

atos

us;

hist

ory

of

rheu

mat

ic

feve

r (?

) at

age

12

15

41

H. P

. fe

mal

e lig

htly

pig

- m

ente

d br

own

brow

n ho

usew

ife

norm

al su

nbur

n re

actio

n; ta

ns

norm

ally

each

June

si

nce

1950

er

ythe

ma

in 2

hour

s max

imum

in

24 h

ours

past

yea

rs, e

ry-

them

atou

s, pa

pu-

lar e

rupt

ion;

pre

s-

ently

, di

scoi

d le

sion

s

form

erly

cle

ared

qu

ickl

y; n

ow

pers

iste

nt

expo

sed

and

Un-

ex

pose

d ar

eas

of

the b

ody

suba

cute

lup

us e

ry-

them

atos

us

16

24

C. B

, fe

mal

e lig

htly

pig

- m

ente

d br

own

haze

l ho

usew

ife

norm

al su

nbur

n re

actio

n; t

ans

norm

ally

each

June

si

nce

1948

er

ythe

ma

in 6

- 24

hou

rs

past

year

s as

abov

e;

pres

ently

disc

rete

er

ythe

niat

ous a

nd

conS

uent

pap

ules

form

erly

clea

red

prom

ptly

; no

w

mos

tly pe

rsis

tent

w

ith s

light

wan

- ta

g

expo

sed

part

s,

face

, "V

" of

nec

k an

d ar

ms

suba

cute

lup

us e

ry-

them

atos

us;

rheu

- m

atic

feve

r (?)

age

15

17

40

A.

V.

mal

e da

rkly

pig

- m

ente

d bl

ack

brow

n ca

rpen

ter

outd

oors

no

rmal

sun

burn

re

actio

n; ta

ns

norm

ally

June

195

1

and

1952

; al

so O

ctob

er

1952

eryt

hem

a in

6 to

24 h

ours

pa

st y

ears

, ery

then

i-

aton

e pa

pula

r er

uptio

n; n

ow p

ap-

ulea

are

dis

cret

e an

d co

nflu

ent

to

form

pla

ques

clea

red

prom

ptly

pr

ior t

o 19

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+

def

inite

(m

arke

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—

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s in

dif

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of

the

sect

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*

In th

e hi

stop

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logi

c dia

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e em

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as d

iscu

ssed

by

Mon

tgom

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(8).

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se a

re n

oted

ab

ove.

The

fin

ding

s w

hich

wer

e co

nsta

nt i

n al

l ca

ses

wer

e hy

perk

erat

osis

, fol

licul

ar p

lugg

ing,

liqu

efac

tion

dege

nera

tion

of th

e ba

sal

cell

c la

yer,

slig

ht to

mar

ked

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vasc

ular

and

per

iapp

endi

geal

infi

ltrat

e, e

dem

a of

the

uppe

r par

t of t

he c

oriu

m (a

lthou

gh n

ot p

rom

inen

t in

case

s 5

and

16),

dila

tatio

n of

the

capi

llari

es o

f the

upp

er p

art o

f th

e co

rium

. and

lac

k of

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itera

tive

chan

ges

in th

e bl

ood

vess

els

of th

e de

eper

pa

rt o

f th

e ce

rium

. Atr

ophy

of t

he r

ete

mal

pigh

ii w

as a

n in

cons

tant

find

ing

and

abse

nt in

cas

es 1

, 2,

and

12;

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ht in

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es 3

and

4;

mod

er-

ate

in c

ase

7; a

nd a

ltern

atin

g w

ith a

reas

of

acan

thos

is in

cas

es 6

and

15. In

cas

es 4

and

5 th

e in

filtr

ate

was

cha

ract

eris

tic in

mos

t par

ts o

f th

e se

ctio

n, b

ut t

here

wer

e sm

all

foca

l ar

eas

of i

nfilt

rate

hug

ging

the

epid

erm

is s

ugge

stiv

e of

atro

phic

lich

en p

lanu

s. T

he c

olla

gen s

how

ed

slig

ht fr

agm

enta

tion

in a

ll th

e se

ctio

ns ex

cept

in c

ase

13 w

here

the

re w

as h

omog

eniz

atio

n an

d m

arke

d fr

agm

enta

tion.

Bas

ophi

lic d

egen

era-

tio

n w

as n

et a

com

mon

fea

ture

.

382 THE JOURNAL OF INVESTIGATIVE DERMATOLOGY

Laboratory studies were done on all patients which included complete bloodcount, urinalysis, sedimentation rate, total protein, albumin-globulin ratio, andserologic test for syphilis. Special laboratory examinations, such as electrocardio-gram, basal metabolism studies, liver and kidney function studies were done whenindicated. L. E. cell determinations were done on 6 patients using the 2 hourblood clot method described by Zimmer and Hargraves (33).

One or more biopsy specimens were taken from the presenting lesions of eachof the patients. In addition, biopsy specimens were removed from the areas whichwere experimentally exposed to 5 minutes and 12 minutes of hot quartz ultra-violet light rays.

TABLE 4Findings in patients with prurigo aestivalis type of polymorphous light eruption (Group I);

patients with subacute lupus erythematosus (Group III); and patients who may repre-sent stages in transition (Group II)

GROUP I GROUP It GROUP III

Relation of eruption to sunlightPast + + +Present +

Persistence of eruptionPast — —Present

Location of eruptionUncovered areas + + +Covered areas —

Laboratory findings —Histopathologic findings consistent with the diag-

nosis of lupus erythematosus + + ++ definite relationship or findings— no relationship or findings

doubtful relationship or findings

Twenty-one patients with skin coloring similar to that of the patients studied,and with minor dermatoses unrelated to exposure to sunlight, served as a controlgroup.

RESULTS

The effects on the patients tested with hot quartz ultraviolet light rays for 10,20, 30, 40, 60 and 120 seconds varied from no reaction from exposures below 40seconds to mild erythema with the longer exposures.* This was similar to resultsobserved in the control group.

Exposures to cold quartz ultraviolet light over 40 seconds, generally resultedin a mild erythema and subsequent slight scaling of the tested areas. Patients inthe control group reacted in a similar fashion.

* The hot quartz mercury vapor burner used in these investigations was in operationfor approximately 180—200 hours. At 75 centimeters, the minimal erythema dose variedfrom 40 seconds to 80 seconds, as determined on a control group of patients.

LUPIJS ERYTHEMATOSUS AND POLYMORPHOUS LIGHT ERUPTIONS 383

Exposures to infra-red rays produced transient erythema. Exposures to Wood'slight rays gave no reaction. These reactions were also similar to those observedin control patients.

Twelve patients (case 1, 2, 6, 7, 9, 11, 12, 13, 14, 15, 16, 17) of the 13 exposedto repeated 5 minute doses of hot quartz ultraviolet light rays, developed anormal sunburn response. The remaining patient (Case 3) developed a folliculo-papular eruption in the test site 4 days after a single exposure. In all 13 patients,histopathologic examination of specimens obtained from the test sites revealedfeatures compatible \vith moderate to severe sunburn reaction, (36), or non-specific dermatitis, dependent on when the biopsy specimen was obtained.

Fourteen patients in the control group who received repeated 5 minute expo-sures of hot quartz ultraviolet light, developed a normal sunburn response. Bi-opsy specimens from four showed non-specific dermatitis.

Seven patients (Cases 3, 6, 11, 12, 14, 15 and 16) of the 9 exposed to a single12 minute dose of hot quartz ultraviolet light rays developed a normal sunburnresponse, the histopathologic features of which were compatible with a diagnosisof severe sunburn reaction or non-specific dermatitis. The remaining 2 patients(Cases 7 and 13) developed an erythematous papular ernption in the test site.Clinically these experimentally reproduced eruptions resembled the presentingclinical eruptions. The details of the latter 2 eases are as follows:

II. P. (Case 7—see figure 1), a white mechanic of 41 years developed, in June 1952, aneruption on the exposed regions of the body (the face, "V" of the neck, and forearms,sharply marginated at the sleeve line). He had had a similar recurrent eruption each summerfor the past 4 years. This disappeared completely in cloudy weather and in the winter.Examination revealed a discrete, light red, urticaria-like, firm, papular inflammatoryeruption on the regions described above. The ernption was diagnosed polymorphous lighteruption of the prurigo aestivalis type. The lesions disappeared in 10 days with adequatesun protection and use of "Sun Screen Derma-pak" (Doak).

Laboratory findings were within normal limits (see chart 2). Histopathologic examina-tion of this presenting eruption was compatible with the diagnosis of chronic lupus cry-thematosus. (Figure 2.)

This patient received a single 12 minute exposure of hot quartz ultraviolet light rays toan uncovered area ("V' of the neck) and to the mid portion of the back. Six hours afterexposure there was marked erythema which reached maximum intensity iu 48 hours. Theerythema remained at maximum intensity for 8 days, and then began to fade. lu its placethere were numerous, firm, elevated, 2 to 4 millimeter sized papules (Figure 3). The histo-logic findings from areas of the experimentally reproduced eruption, from both the exposedas well as the protected sites, revealed features suggestive of chronic lupus erythematosus(Figure 4). The papular eruption disappeared completely 14 days after its appearance.

G. C. (Case 13—see figure 5), a white housewife of 42 years has had, since the age of 23, atransient eruption which recurred ia the late spring or early summer after sun exposure.These eruptions cleared, within 2 to 3 weeks after onset, with sun protective measures.On June 23rd, 1952, several days after exposure to the bright midday sun, the patient notedan eruption on the uncovered parts of the body ("V" of neck, face, forehead, and arms.)There were erythematous, inflammatory, elevated, firm, discrete papules. With coalescence,these lesions formed sharply demarcated plaques in a butterfly distributiou over the fore-head, infraorbital, and malar regions.

On physical examination the heart was found to be slightly enlarged to the left with ac-centuation of the aortic second sound. The blood pressure was 190/100 (in spite of a lumbarsympathectomy in 1947). The heart sounds were of good quality and the lungs were clear.

FIG. 1. R. P. Case 7, illustrating the typical inflammatory, papular eruption over thesides of the face and neck following exposure to sunlight.

FIG. 2. Biopsy specimen from clinical eruption of patient R. P., Case 7, showing hyper-keratosis, follicular plugging, moderate atrophy of the rete malpighii, liquefaction de-generation of the basal cell layer, edema of the upper portion of the corium, dilatationof capillaries, and marked, patchy perivascular and periappendigeal lymphocytic infiltrate.

384

rA

FIG. 3. Lesions of polymorphous light eruption reproduced experimentally on "V" ofneck of patient 11. P., Case 7. Photograph taken 10 days after single 12 minute exposure tohot quartz ultraviolet lamp placed with burner 75 cm. from skin.

FIG. 4. Histopathologic specimen from eruption reproduced experimentally on normalskin of mid-back in patient H. P., Case 7, reveals hyperkeratosis, with follicular plugging,liquefaction degeneration of the basal cell layer, edema of the upper portion of the eorium,marked perivaseular and periappendigeal infiltrate. The rete malpigbii in this section doesnot show atrophy.

385

A...SeJ. ____

4

'. delt.

—J

386 THE JOURNAL OF INVESTIGATIVE DERMATOLOGY

FIG. 5. C. C. Case 13, illustrating the clinical appearance of the papular type of poly-morphous light eruption in which confluence of lesions resulted in a clinical picture resem-bling subacute lupns erythematosus. Fifteen days after onset, with the use of adequate sunprotection, the eruption had completely disappeared.

FIG. 6. C. C. Case 13. Histopathology of the clinical eruption showing hyperkeratosis,follicular plugging, marked atrophy of rete malpighii, liquefaction degeneration of thebasal cell layer, slight perivascular and periappendigeal lymphocytic infiltrate, markededema of the upper part of the corium, and dilatation of the capillaries. These features arecompatible with the diagnosis of subacute lupus erythematosus.

FIG. 7. C. G. Case 13. Lesions of the papular type of eruption reproduced experimentallyon normal skin of "V" of neck as it appeared 15 days after a single 12 minute exposure tohot quartz ultraviolet lamp placed with burner 75 cm. from skin.

FIG. 8. C. C. Case 13. Histopathology of eruption experimentally reproduced on normalskin of mid-hack after single 12 minute exposure to hot quartz lamp. There is markedhyperkeratosis, follicular plugging, extreme atrophy of rete malpighii, liquefaction de-generation of basal cell layer, marked edema of the upper part of the corium with capil-lary dilatation, and perivascular and periappendigeal lymphocytic infiltrate. Note plug-ging of the sweat pore. These features are compatible with the diagnosis of subacutelupus erythematosus.

387

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388 THE JOURNAL OF INVESTIGATIVE DERMATOLOGY

Clinically the eruption resembled both polymorphous light eruption of the prurigoaestivalis type, and subacute lupus erythematosus.

Except for a sedimentation rate of 24 mm/hour, the laboratory findings were normal(see chart 2). Histologically the section showed features compatible with the diagnosis ofsubacute lupus erythematosus (see figure 6).

The lesions disappeared almost completely in 15 days with adequate sun protection andthe use of "A-flu" cream (Texas Pharmacal Company).

This patient received a single 12 minute exposure of hot quartz ultraviolet light raysto an uncovered ("V" of the neck) and covered (mid-back) portion of the body. Six hoursafter exposure, erythema was first noted. This reached maximum intensity in 48 hours.The erythema persisted at this level for 10 days, after which it began to fade. On the 15thday after exposure definite, firm, elevated, discrete, inflammatory papules, 2 to 4 millimetersin diameter, appeared in the areas (see figure 7). Biopsy specimens from the areas of ex-perimentally reproduced eruption (figure 8) showed findings compatible with the diagnosisof subacute lupus erythematosus. Twenty-eight days after exposure, the erythema hadcompletely subsided, but the papules had not as yet completely disappeared.

Fourteeu patients in the control group received a single exposure of hot quartzultraviolet light for 12 minutes, and developed a normal sunburn response. Noneof them developed a papular eruption in the test site even after several weeksobservation. Biopsy specimens of 6 of these patients showed non-specific der-matitis.

DISCUSSION

The 9 patients in group I have lesions which are of the prurigo aestivalis typeof polymorphous light eruption. These patients develop lesions despite the factthat they acquire a normal tan to sunlight. Urticarial lesions were noted at theonset of the eruption in 3 of the patients (cases 1, 12, and 13). According to Blum(5) this probably indicates some derangement in the sunburn mechanism whichis different from true urticaria photogenica.

There is a wide quantitative variation in normal reactivity to sunburn radia-tion. Polymorphous light eruptions, on the other hand, are abnormal responsesin that they are qualitatively different from those of the normal sunburn reaction(5). In our cases of the experimentally reproduced eruption, the papules devel-oped only in an area of intense erythema, and this erythema differed qualitativelyfrom that seen in controls in the following manner: (a) delay in production ofmaximum erythema. Maximum erythema developed in both patients (cases 7and 13) in 48 hours, as compared to 6 to 24 hours in controls. (b) persistenceof maximum erythema for 8 days in patient 7, and 10 days in patient 13.The erythema began to fade within 24 to 48 hours in controls. (c) greater inten-sity of erythema as compared to controls. (d) appearance of inflammatorypapules in the test area as the maximum erythema begins to fade. (e) failure ofresidual pigmentation to appear. In control patients as the erythema fades it isreplaced by pigmentation and scaling; no papules are seen even after prolongedobservation. Gilfillaa (9) reports a case in which exposure to ultraviolet hotquartz mercury vapor rays to an area over the sternum for 2 minutes at 15inches produced vivid erythema which began to fade after 4 weeks and leftpapules in the test site. Epstein (2) reports several cases in which he reproduced

LTJPUS ERYTHEMATOSUS AND POLYMORPHOUS LIGHT ERUPTIONS 389

experimentally a vivid erythema in the test site followed by a delayed papulareruption. He feels that the provocation of lesions of prurigo aestivalis appearsonly when marked radiation erythema is produced, regardless of the type ofirradiation. We were able to elicit this same type of inflammatory papular re-sponse in 2 of our 9 patients experimentally exposed to the sunburn spectrum for12 minutes. Other investigators have also been able to reproduce this responsewith the sunburn spectrum (9, 11, 12).

Several reasons may be advanced for failure to reproduce the eruption experi-mentally in the other 7 patients tested with the sunburn spectrum: (a) Otherinvestigators have been able to reproduce this eruption using light sources withwave lengths over 3200 A (5, 13, 14, 15): (b) the patients' sensitivity may havebeen lost or destroyed, or the test site may not have been sensitive at thetime of the test. These periods of temporary refractoriness may alternate withphases of "sensitization" and, therefore, one is unable to reproduce the erup-tion during these refractory periods (2, 5, 16): (c) the light rays may not havebeen of sufficient intensity to elicit an adequate response. It would appear thatthe threshold for production of erythemato-papular lesions is higher th n forproduction of erythema (5). Thus, we were able to produce erythema but wereunable to elicit the papular response with repeated 5 minute exposures of hotquartz ultraviolet light rays. A single 12 minute exposure (i.e. greater inten-sity) resulted in reproduction of the eruption in 2 cases. Touraine and Dup-perat (12) were able to reproduce an eruption with 2 minutes contact expo-sure but were unable to do so using a 4 minute exposure at 50 centimeters witha Kromayer lamp. Epstein (2) also noted that he was able to produce pruri-go-like papules at sites of longer exposure to light and was not able to reproducethis in the same individual with shorter exposures. It is noted clinically thatpatients develop their eruptions of prurigo aestivalis in spring and early summerwhen the rays of the sun reaching the earth are at their greatest intensity in thislocality: (d) the patient may be sensitive to a particular wave length not pro-duced by the line type hot quartz mercury arc lamp used in this investigation,but produced by the sun which has a continuous spectral emission. Thus thespecific spectral band needed to elicit this erythemato-papular eruption experi-mentally in sensitive patients (see table 1) may not have been emitted in thelight sources available to us (14).

The qualitative differences in response to sunlight and the possible alternatingphases of "sensitization" and refractoriness suggest an allergic factor in prurigoaestivalis (2, 5, 6, 17, 18, 19, 20, 21). Epstein (2) hypothesizes the transformationof proantigen, a precursor substance present in the skin of a photoallergic person,to antigen under the influence of irradiation.

Lupus erythematosus may also represent an allergic response to light (21,22, 23). Stokes and his coworkers (6) point to the work of Rost and Baccareddato support the concept of a vasculo-allergic reaction in the production of lupuserythematosus. Hargraves (34) offers the hypothesis that anaphylactic reactionsin areas of disintegration of platelets might give rise to the vascular lesions ofdisseminated lupus erythematosus. He states that this possibility seems more

390 TEE JOURNAL OF INVESTIGATIVE DERMATOLOGY

likely when it is recalled that most patients with this disease have an "allergicmake up, frequently display an untoward reaction to drugs, are prone to reactunfavorably to blood transfusions, are likely to be sensitive to the sun, andoften have hay fever and urticaria". On the other hand, Klemperer, Pollack,and Baehr are reluctant to accept the role of allergy in lupus erythematosus(24).

The relationship between light and the production and exacerbation of lupuserythematosus is well known. In a series of 12 cases of acute disseminated lupuserythematosus, Rose and Pillsbury (25) found that 6 of their patients "developedtheir initial eruption or a cutaneous exacerbation immediately after exposureto the sun or ultraviolet light. Three patients gave histories indicating abnormalsensitivity to sunlight for months or years prior to the onset of the acute phaseof their disease." Other investigators have also pointed out the relationshipbetween lupus erythematosus and light (26, 27, 28, 29, 30).

All patients included in this study had a definite history of altered sensitivityto sunlight for a number of years, characterized by recurrent, transient, erythe-mato-papular eruptions on uncovered areas. The patients in Group I (trueprurigo aestivalis) reported a definite outbreak after exposure to sunlight inthe spring and early summer and not at other times of the year. On the otherhand, the patients in Group III had subacute lupus erythematosus and althoughtheir past history began like that of prurigo aestivalis (Group I), the processgradually changed to one characterized by (a) persistence of lesions in the ab-sence of light exposure (i.e. no longer transient); (b) new outbreaks at any timeof the year with no apparent relationship to exposure to sunlight; (c) the oc-currence of lesions on covered as well as uncovered areas. The patients in GroupII have eruptions that represent varying stages in transition between GroupsI and III (see table 4).

Our 17 patients presented histopathologic findings consistent with the diagno-sis of lupus erythematosus in most instances. Lamb and his coworkers (4) de-scribed histopathologic findings on the specimens removed from their 24 patientswith the plaque-like or the erythema perstans solare type of polymorphous lighteruption which showed many of the features observed in lupus erythematosus.Clinically too, our patients showed a striking resemblance to lupus erythemato-sus, and several in Groups I and II had been treated by competent observersfor this disease. There are references to the rapid remission of some cases of"subacute lupus erythematosus" with the use of a sunscreen preparation (31),nicotinamide (32), gold, bismuth, liver extract, etc. Some of these patients mayhave been similar to patients whom we placed in Group I or Group II. It hasbeen noted (2, 4) that differentiation of the evanescent, multiform type of lupuserythematosus from the prurigo type of polymorphous light eruption may bedifficult clinically, and laboratory studies may be necessary to help clarify thesituation. Sellei and Liebner (11) have noted an increased sedimentation rate insome of their cases of prurigo aestivalis. Thus it appears impossible in someinstances to distinguish prurigo aestivalis from subacute lupus erythematosusboth clinically and histologically.

LUPIJS ERYTHEMATOSUS AND POLYMORPHOUS LIGHT ERUPTIONS 391

SUMMARY AND CONCLUSIONS

(1) This study suggests a possible relationship between an altered reaction tosunlight, as manifested by the prurigo type of polymorphous light eruption, andsubacute lupus erythematosus. The 17 patients investigated gave a definitehistory of altered sensitivity to sunlight characterized by development of re-current, transient, erythemato-papular eruptions on uncovered areas for a numberof years directly related to sunlight exposure.

(2) The reactions shown by these 17 patients when exposed to doses of ultra-violet light rays less than 120 seconds delivered by a cold quartz apparatus anda hot quartz apparatus, to infra red rays and Wood's light rays, were similar tothose shown by patients in a control group.

(3) Thirteen patients were exposed to ultraviolet light rays generated by ahot quartz air-cooled mercury vapor burner for 5 minutes at a distance of 75centimeters from a 2.5 centimeter square test site, both on covered and uncoveredparts of the body (mid portion of the back and "V" of the neck respectively).This exposure was repeated at intervals of one week for 5 to 10 weeks using thesame test site. In no instance was a papular eruption produced which wouldcorrespond to the presenting eruption. Biopsy specimens taken from thesetested areas revealed non-specific dermatitis. The same results were obtainedin control patients.

(4) Nine patients with polymorphous light eruption received a single exposureof hot quartz ultraviolet light rays for 12 minutes at 75 centimeters from a 5centimeter square area, to covered and uncovered areas. Two developed intenseerythema and a delayed papular eruption in the test site which resembled thepresenting eruption clinically. Morphologically and histologically, the lesionswere indistinguishable from lupus erythematosus. This experimentally repro-duced eruption was qualitatively different from the normal sunburn response,as observed both in the control patients and in the other 7 patients tested.

(5) The histopathologic findings in the specimens from the 17 patients studiedwere consistent with the diagnosis of lupus erythematosus. Also, at its height,the eruption in most cases resembled subacute lupus erythematosus. In addition,a number had laboratory findings usually associated with systemic activity asfound in this disease.

(6) On the basis of clinical, laboratory and histopathologic findings, it wasfelt that our cases represented 3 closely related groups:

Group I, 9 patients with prurigo aestivalis, with a definite recurrence oferuption after exposure to sunlight in spring and summer, and not at other timesof the year. These patients showed no clinical or laboratory evidences of systemicdisease.

Group III, 4 patients with subacute lupus erythematosus, but with a pasthistory suggesting prurigo aestivalis.

Group II, 4 patients who represented varying stages in transition betweenGroups I and III.

(7) Therefore, it would appear from our studies, that lupus erythematosus is

392 THE JOURNAL OF INVESTIGATIVE DERMATOLOGY

not a clear-cut concept and the reaction to light rays in our patients may repre-sent stages in transition from subclinical (or latent) to manifest clinical systemiclupus erythematosus.

REFERENCES

1. HAIJSMANN, W., AND HAXTIIAUSEN, H.: Die Lichterkrankungen der Haut: Strahlen-therapie; Sonderbände, Berlin, Urban and Schwarzenberg, Vol. XI, 1929.

2. EPSTEIN, S.: Studies in Abnormal Human Sensitivity to Light, I Prurigo Aestivalis,Eczema Solare and Urticaria Photogenica; II Light Sensitivity in Prurigo Aestivalis,Eczema Solare and Urticaria Photogenica; III Passive Transfer of Light Hyper-sensitivity in Prurigo Aestivalis; IV Photoallergic Concept of Prurigo Aestivalis,J. Invest. Dermat. 5: 187—196, 225—241, 285—287, 289—295, 1942.

3. Hurmrr.soi, J.: A Case of Summer Eruption Recurring with Great Severity for ManyYears but Finally getting Well (a form of Kaposi's disease), Tr. Clin. Soc. Lond.22: 80—83, 1889.

4. LusB, J. H., SHELMIRE, B., COOPER, Z., MORGAN, R. H., AND KEATY, C.: Solar Derma-titis, Arch. Dermat and Syph. 62: 1—27, 1950.

5. Bxims, H. F.: Photodynamic Action and Diseases Caused by Light, American ChemicalSociety Monograph Series, No. 85, New York, Reinhold Publishing Corporation,1941.

6. SToKES, J. Hq BEERMAN, H., AND INGRAHAM, N. H., Ju.: Photodynamic Effects inDermatology, Am. J. M. Sc. 203: 608—623 (Part I); 204: 601—624 (Part II), 1942.

7. STOKES, J. H., AND CALLAWAY, J. L.: Pyogenic Relapse and Sensitiveness to Light inCertain Dermatoses; Influence of Factors of Intercurrent Infection, Arch. Dermat.and Syph. 36: 976—986, 1937.

8. MONTGOMERY, H.: Pathology of Lupus Erythematosus, J. Invest. Dermat. 2: 343—359,1939.

9. GILI"ILLAN, W.: Clinical Note; Rasch's Polymorphic Light Eruption, Brit. J. Dermat.49: 241—242, 1937.

10. KESTEN, B. M., AND SLATHIN, M.: Diseases Related to Light Sensitivity, Arch. Dermat.and Syph. 67: 284—301, 1953.

11. Sam, J., AND LIEBNER, E.: Vber Prurigo Aestivalis, Arch. f. Dermat. u. Syph. 152:19—33, 1926.

12. TOIIRAINE, A., AND DUPPERAT: "Summer Prurigo." Bull. Soc. franc, de Dermat. etSyph. 42: 913—916, 1935.

13. SCBAUMANN, J., AND LINDHOLM, F.: Etudes cliniques, actinobiologiques et histologiquessue le summer prurigo d'Hutchinson, Ann. Inst. d'actinol. 6: 93—100, 1932; Fort-gesetzte aktinobiologische Untersuchungen Hutchinson's Sominerprurigo,Strahientherapie. 61: 646—648, 1938.

14. TURNER, W. J.: Chronic Polymorphous Light Dermatitis with Report of a Case, M.Bull. Vet. Admin. 15: 270—276, 1939.

15. URBACH, E., AND KONBAD, J.: tber eine durch den langwelligen Anteil des Sonnenspekt-rums Erzeugte Lichtdermatose vom Typus der Prurigo aestivalis Hutchinson. DieLichtschutzende Wirkung des Resorcin, Strahientherapie 32: 193—204, 1929.

16. TEMPLETON, H. J., AND LUNSFORD, C. J.: Eczema Solare and Porphyria, Arch. Dermat.and Syph. 25: 691—703, 1932.

17. CALLAWAY, J. L.: Passive Transfer of Light Sensitivity, Arch. Dermat. and Syph.41: 889—891, 1940.

18. MtHLMANN, J., AND AXOBJAN, A.: Experimenteller Beitrag zur Aetiologie der PrurigoAestivali, Arch. f. Dermat. u. Syph. 159: 318—323, 1930.

19. DTJKE, W. W.: Physical Allergy as a Cause of Dermatoses, Arch. Dermat. and Syph.13: 176—186, 1928.

LUPTJS ERYTHEMATOSUS AND POLYMORPHOUS LIGHT ERUPTIONS 393

20. BERNSTEIN, F.: Beiträge zu den physikalischen Idiosynkrasien der Haut, Arch. f.Dermat. u. Syph. 168: 177—182, 1933.

21. JAU5I0N, H., AND PAGS, F.: Les Maladies de Lumiere et leur traitment; Chapter 9:Les actino-anaphylactoses, pg. 122—174, Masson et Cie, Paris, 1933.

22. EPSTEIN, S.: Photoallergy and primary photosensitivity to Sulfanilamide, J. Invest.Dermat. 2: 43—51, 1939; Allergische Lichtdermatosen, Dermatologica. 80: 291—320,1939.

23. Fox, R. A.: Allergic Aspect of Lupus Erythematosus, Arch. Path. 36: 311—315, 1943.24. KLEMPERER, P., POLLACK, A. D., AND BARER, G.: Diffuse Collagen Disease, J. A. M. A.

119: 331—332, 1942.25. ROSE, E., AND PILLSBURY, D. M.: Acute Disseminated Lupus Erythematosus—A

Systemic Disease, Ann. mt. Med. 12: 951—963, 1939.26. FElT, H.: Light as an exciting Agent in Lupus Erythematosus and Other Dermatoses,

J. M. Soc. New Jersey. 24: 226—228, 1927.27. BRAIN, R. T.: Lupus Erythematosus Replacing the Lesions of Erythema Solare, Proc.

Roy. Soc. Med. 26: 748—749, 1933.28. BECHET, P. E.: Excessive Solar and Phototherapeutic Irradiation, A Causative Factor

in Certain Diseases of the Skin, Arch. Dermat. and Syph. 29: 221—227, 1934.29. RASCH, C.: Effect of Light on Skin and Skin Diseases, Proc. Roy. Soc. Med. (Sect.

Dermat.). 20: 1—20, 1926.30. MACKEE, G. M.: Ultraviolet Light Therapy in Dermatology, J. A. M. A. 98: 1553—1561

(April 30), 1932.31. G0UGER0T, H., BURNIER, R., AND ALBEAIIX-FERNET, M.: Lupus érythémateux des

parties découvertes a type pellagro!de déclenché par un coup de soleil, Bull. Soc.franc. de Dermat. et Syph. 41: 280—285, 1934.

32. CIVATrE, A., TZANCK, A., AND Sun, E.: Dermatite simulant le lupus érythémateuxsubaigu. Traitment par l'acide Nicotinique, Bull. Soc. franc de Dermat. et Syph.46: 1344—1346, 1939.

33. ZIMMER, F. E., AND HARORAVES, M. M.: The Effect of Blood Coagulation on L. E.Cell Formation, Proc. Staff Meet. Mayo Clin. 27: 424—430, 1952.

34. HARGEAVES, M. M.: The L. E. Cell Phenomenon, Proc. Staff Meet. Mayo Clin. 27:419-423, 1952.

35. MICHELSON, H. E.: Remarks on Lupus Erythematosus, Proc. Staff Meet. Mayo Clin.27: 431—436, 1952.

36. PERCIVAL, G. H., DRENNAN, A. M., AND DODDS, T. C.: Atlas of Histopathology of theSkin, Baltimore, Williams and Wilkins, 1947, pp. 209—213.

DISCUSSION

DR. STEPHEN ROTHMAN, Chicago, Ill.: This contribution is highly significantfrom both the experimental and clinical points of view. I would like to ask whatthe ultraviolet dosage was in terms of threshold erythema doses in the tests. Atpresent this is the only way to express ultraviolet dosages to make them repro-ducible with some degree of accuracy. Also I would like to know what was meantby "adequate sun protection". Did you protect against the 3000 Angstrom range,the 4000 Angstrom range or against the whole ultraviolet spectrum? In ourexperience the provocative effect of sunshine in lupus erythematodes is a functionof the sunburn range in the region of 3000 Angstrom, and p-aminobenzoic acidwhich filters out this range gives complete protection.

I was most impressed with the last slide showing the reaction to the ultraviolettesting. The reaction seemed to be identical with a patch of chronic lupus erythe-

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matodes. I even thought it possible to recognize follicular plugging. The authorssucceeded in demonstrating that cases of "polymorphous light eruptions" oftenare cases of subclinical lupus erythematodes. Maybe this is always the case.

DR. H. MONTGOMERY, Rochester, Minn.: I had the opportunity, through thecourtesy of Zola Cooper, of studying a series of histologic cases of solar dermatitisof various types,1 and there were some cases where the question arose of lupuserythematosus histologically and Dr. Cooper and I went over those slides per-sonally and most of those slides did not show the liquefaction degeneration oras much atrophy, or plugging of the epidermis as we see in lupus erythematosus.

In previous studies that I made with Dr. Goeckerman and those made withDr. McCreight (Arch. Dermat. and Syph. 61: 1 Jan. 1950), in a large series ofcases of lupus erythematosus of all types, one usually does not find histologicfeatures of lupus erythematosus before the fourth to sixth week. Therefore ifone were to perform biopsies 15 or 20 days after ultraviolet irradiation, the pre-senters are probably justified in saying that there are beginning signs of lupuserythematosus but in at least one or two of the slides, as I glanced at them, asthey were shown I would not have been willing to say lupus erythematosus.Distinction from solar dermatitis of different types calls for biopsy at a laterdate. There are thin-skinned, often red-headed individuals, whose skin is atrophicto start out with. Correlation of clinical and histopathologic findings is funda-mental for accurate diagnosis.

DR. MARION B. SULZBERGER, New York, N. Y.: First I would like to expressmy congratulations to Drs. Cahn, Levy, Shaffer and Beerman for this funda-mental and interesting piece of work, interesting both practically and theoreti-cally.

One question I would like to ask is whether they have studied the possibilityof this final clinical and histologic picture at the site of the damage from ultra-violet light being a form of isomorphic reaction due to non-specific local irrita-tion—in other words the Koebner phenomenon. Have they tried damaging theskin in these patients with irritating measures other than light?

The second question I would like to ask is whether they have tried treatingthese cases with atabrine, chloroquine or drugs of that series? There is now inpress in the Journal of Investigative Dermatology a paper by Harvey and Coch-ran of Glasgow which I was privileged to see. These authors carried out a studywith atabrine and chioroquine, with bismuth and other preparations, includingother antimalarials in lupus erythematosus. Their report is not the only reportwhich indicates that the anti-lupus erythematosus effect of atabrine and drugsof the fiavine series does not depend upon their antiplasmodial action. In otherwords, there are some powerful anti-plasmodial drugs which are apparently notat all effective in the treatment of lupus erythematosus; so it appears that itmust be some other properties, other effects or functions of the atabrine or the

'Lamb, J. H.; Shelmire, B.; Cooper, Z.; Morgan, R. J.; and Kentz, C. Arch. Dermat.and Syph. 62:1,1950.

LUPTJS ERYTHEMATOSUS AND POLYMORPHOUS LIGHT ERUPTIONS 395

atabrine series of drugs which exert the benefits in lupus erythematosus. Ourexperiences with members of the armed forces exposed to the sun in the SouthPacific as well as some experimental data of our group at the New YorkSkin and Cancer Unit suggest that these drugs may act by deposit in the surfaceepithelial layers and thus function as sun screening agents. At any rate it wouldbe interesting and valuable practically to study the sun-screening effects inthese patients systemically treated with atabrine and drugs of that series.

DR. ALFRED HOLLANDER, Springfield, Mass.: I would like to ask the pre-senters how much experience they have had with cases of chronic discoid lupuserythematosus of long standing concerning the exposure to sunlight. I ask thisquestion because I believe that many of us have observed a great number ofcases which were continuously exposed to sunlight or were treated locally withthe Kromayer lamp, and although they were exposed no flare-ups developed.

Is it true that we have to differentiate chronic discoid lupus erythematosus oflong standing from acute and subacute lupus erythematosus as far as the danger-ous effects of exposure to sunlight are concerned?

DR. BamIcE M. KESTEN, New York City, N. Y.: Our index of suspicion isdefinitely heightened by work of this kind.

I too would like Dr. Cahn's opinion on whether individuals with so-calledlupus erythematosus should be protected from the sun? And is he hesitant intesting individuals with any type of lupus erythematosus to wave lengths foundin sunlight?

DR. EDWIN J. LEVY (in closing): I wish to thank the discussers for theirexcellent analysis of our work.

Dr. Rothman asked the dosage of ultraviolet light required to reproduce theeruptions. Approximately eight to ten times the erythema dose was required.It is a known fact that the intensity of light needed for the production ofpolymorphous light eruption is much greater than that required for productionof simple erythema.

Dr. Rothman asked about the use of p-aminobenzoic acid as a sun screen.We prescribed p-aminobenzoic acid in our cases as recommended by Dr. Roth-man in his article in 1942. We found that it is an excellent sun screen, and usingthis preparation we have succeeded in suppressing the eruption in all cases inGroup I, the so-called polymorphous light eruption.

Our findings indicate that the sunburn spectrum was responsible for poly-morphous light eruption in our cases. However, there are numerous reports inthe literature by authors who have reproduced this eruption using other spectralbands.

Dr. Montgomery noted that it requires several weeks for the development ofthe typical histologic picture of lupus erythematosus. We have, on the basis ofour histologic studies, also come to this conclusion. In our cases, many of thebiopsies were taken three to fifteen days after the appearance of the eruption and

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showed features "suggestive of lupus erythematosus", although some werereported unequivocally, lupus erythematosus. I might add that the slides wereread objectively by Dr. Beerman. The slides were coded and submitted to himas unknowns.

Dr. Sulzberger inquired about the use of atabrine and chloroquine in suppres-sion of solar dermatitis. We are currently testing a group of these patients withatabrine, but are not yet ready to report conclusions. We can state, however, thatthose cases in Group II and III improved on atabrine.

In answer to Dr. Kesten, we have had some hesitancy in indiscriminitely test-ing these cases to light. One of the cases that we placed between Groups II andIII, that is, between the transition group and subacute lupus erythematosuscategory, after sun testing did develop acute systemic lupus erythematosus. Wefeel that we may have been responsible for this exacerbation.

The question was asked by Dr. Hollander regarding the degree of light sensi-tivity in long standing cases of chronic discoid lupus erythematosus It is ourimpression that not all cases of chronic discoid lupus erythematosus are lightsensitive. We have seen some of our cases in Groups II and III with the featuresof subacute lupus erythematosus, having lesions highly suggestive, also, ofchronic discoid lupus erythematosus. These cases, in addition, give a definitepresent or past history of sensitivity to sunlight, with exacerbation or reproduc-tion of their eruption upon exposure to light.