JEADV (2002) 16, 193 206

C M E A RT I C L E

Polymorphous light eruption

Blackwell Science Ltd

AJ Stratigos, C Antoniou, AD Katsambas*

Department of Dermatology, University of Athens School of Medicine, Andreas Sygros Hospital for Skin and Venereal Diseases, 5 Dragoumi Street,
Kesariani 161 21, Athens, Greece. *Corresponding author, E-mail: phbiolun@otenet.gr

AB S T R A C T
Polymorphous light eruption (PLE) is a common idiopathic photosensitivity disorder with an estimated
prevalence of 1020%. It is characterized by an intermittent skin reaction to ultraviolet (UV) radiation
exposure, consisting of non-scarring pruritic erythematous papules, vesicles or plaques that develop on light-
exposed skin. Despite the different morphology in different individuals, the eruption tends to have a mono-
morphous presentation in any single subject. The histopathological features of PLE are distinct and comprise
a perivascular lymphocytic infiltrate in the dermis, subepidermal oedema and variable epidermal changes.
The pathogenesis of PLE is not well known, but findings suggest that it is a delayed-type hypersensitivity
reaction to one or more UV-modified cutaneous antigens. The principal action of PLE is mainly in the UVA
region, although some subjects exhibit sensitivity to UVB alone or to both UVA and UVB radiation at the same
time. Preventive measures in PLE include the regular use of photoprotective methods combined with graduated
exposures to natural sunlight. The induction of immune tolerance by phototherapy and photochemotherapy
are useful prophylactic methods in moderate to severe cases. The role of systemic agents in the management
of PLE is under investigation. This article reviews the epidemiological, pathogenetic and clinical aspects of
PLE and discusses recent advances in the diagnostic approach and management of this condition.
Key words: photoprovocation, photosensitivity, phototesting, phototherapy, polymorphous light eruption,
psoralen + ultraviolet A, ultraviolet radiation, ultraviolet A, ultraviolet B

Received: 23 January 2001, accepted 6 December 2001

number of light-induced dermatoses, e.g. PLE, solar urticaria,

Introduction
Perhaps the first to recognize the features of polymorphous
light eruption (PLE) was Robert Willan, who at the end of the
eighteenth century used the term eczema solare to describe a
skin rash that had manifested after exposure to sunlight.1 A
century later, Sir Jonathan Hutchinson described 14 patients,
mostly young women, who presented with an intermittent
papular eruption on the face and arms during the spring and
early summer.2 The covered areas of the body were also involved
in some patients and scarring was occasionally present. He
named this eruption summer prurigo, a term that probably
encompassed various photosensitive conditions (PLE, hydroa
vacciniforme and actinic prurigo). Other synonymous terms,
such as prurigo adolescentium, prurigo aestivalis and acne
prurigo were also used to describe light-induced rashes. The
specific term polymorphous light eruption was first used
by Rasch in 1900 and was further defined by the work of
Haxthausen.3,4 At that time, the designation of PLE included a
actinic prurigo, chronic actinic dermatitis, cutaneous lupus
erythematosus and porphyria. In the following decades, these
photodermatoses were more clearly defined and PLE was
eventually recognized as a specific, individual clinical entity.
The interindividual polymorphism of lesions, however, and the
disparities in the action spectrum involved suggest that PLE
may still encompass more than one photosensitivity disorders,
each with a different pathogenetic mechanism. In addition,
several questions remain to be answered regarding the aetiology
and pathogenesis of the disease, its prevention and manage-
ment, and its relationship with other photo-induced entities, in
particular actinic prurigo, inherited PLE in American Indians,
and certain subtypes of lupus erythematosus.
Epidemiology
PLE is considered the most common of the idiopathic photo-
dermatoses. It comprises more than 90% of all photo-induced

2002 European Academy of Dermatology and Venereology 193

194 Stratigos et al.
eruptions, with a prevalence that is estimated as ranging from
10 to 20% of the population and possibly even higher, as many
cases of mild intensity are tolerated well by affected individuals
and do not require medical attention.5,6 In an interview of 271
apparently healthy consecutive entrants to a medical library in
Boston (MA, USA), 10% gave a history of symptoms consistent
with PLE6 and in a survey of 412 employees of a Scandinavian
pharmaceutical company, 21% reported symptoms suggestive
of the condition.7
PLE has a wide geographical distribution and is seen more
frequently in temperate than tropical areas. The incidence rate
of PLE in the UK, for instance, is approximately 15% compared
with 5% in Australia, a difference that may arise from the
different ultraviolet (UV) A and UVB concentrations in these
geographical regions.8
PLE can affect all races and skin types, although it appears
more frequently in fair-skinned individuals; in fact it has been
reported also in black and Oriental people, and American
Indians.9,10 Women are affected two to three times more often
than males, although some investigators have reported an even
higher female to male ratio (7 : 1).5,11,12 This preponderance of
women implies that PLE involves a hormonal mechanism, but
this hypothesis has not been proven. The age of onset ranges
from childhood to late adult life, with the majority of patients
experiencing their first attack by the age of 30 years.12,13 Onset
during childhood (age < 10 years) is seen less often in PLE (20%
of patients) than in actinic prurigo (86%).12 Recent changes in
sun exposure habits, however, may contribute to the increased
frequency of childhood involvement that has been lately
reported.11
A positive family history is reported in 356% of PLE patients,
but the genetics of the disorder are not well understood.7,14
A familial type of PLE seen in American Indians is inherited
through an autosomal dominant mode with reduced pene-
trance and probably represents a form of actinic prurigo.15 HLA
phenotyping in these subjects revealed a positive association
with HLA types A24 and Cw4 and a negative association with
type A3.16 This contrasts with the finding for PLE, where HLA
typing did not show any significant differences between affected
individuals and control populations.17
Aetiology and pathogenesis
The aetiology of PLE is unknown, in spite of numerous
hypotheses and speculations that have been suggested. Most
of these have focused on the existence of an endogenous or
exogenous photosensitizer that initiates the reaction. A
disturbance in tryptophan metabolism with the accumulation
of cynurenic acid was initially proposed.18 Although this theory
has not been confirmed, it constituted the basis for the thera-
peutic use of nicotinamide in PLE.19 A hormonal imbalance has
also been speculated in PLE, given the predominance of female
patients, and, at some point, oral contraceptives were implicated
2002 European Academy of Dermatology and Venereology JEADV (2002) 16, 193 206
as potential photosensitizers, a notion that has been disproven.20
In addition, the release of leukotriene B4 from leucocytes of
PLE subjects after UVA radiation has been reported but no
conclusive interpretation of this finding has been offered.21
At present, PLE is considered to be an abnormal immune
response to sunlight, which is further influenced by genetic,
photobiological and biochemical factors.
Genetic aspects
A family history of PLE has been reported in up to 50% of
subjects in some series.14 Until recently, however, it was not
clear whether these familial cases of PLE indicate a possible
genetic basis of the disease or whether they simply represent
the occurrence of familial clustering as a result of shared
environmental background. In a recent study, Millard et al.
used the twin model to address the question of PLE heritability.22
In 420 pairs of adult female twins (119 monozygotic and 301
dizygotic twins), assessed by a quantitative genetic model, the
prevalence of PLE was 21% and 18% in monozygotic and
dizygotic twins, respectively, with a higher probandwise con-
cordance for the disease in monozygotic than dizygotic twin
pairs. In addition, a family history of PLE in first degree-
relatives was present in 12% of affected twins, compared with 4%
of unaffected twin pairs. These data substantiate the possibility
of genetic susceptibility in PLE and demonstrate that the disease
is multifactorial, comprising genetic and unique environmental
components. McGregor et al. approached the same question by
using segregation analysis to assess the heritability of photo-
sensitivity in 420 individuals of PLE and actinic prurigo-
ascertained families.23 Across 58 pedigrees, the prevalence of
photosensitivity (predominately PLE) in first degree relatives of
patients with PLE and actinic prurigo was 20.9% and 23.7%,
respectively, compared with a prevalence rate of 13.6% in
the general population. Genetic modelling for PLE across all
pedigrees revealed a dominant mixed mode of inheritance
implying the presence of a high frequency, low penetrance
susceptibility allele. It was estimated that up to 72% of the UK
population carry this PLE susceptibility allele, but only 24% of
susceptible females and 13% of susceptible males will manifest
the affection. The expression of the disease in these individuals
is largely determined by a polygenic component with an
additional environmental component, possibly exposure to
sunlight. These data also support the hypothesis that PLE and
actinic prurigo share a common genetic background, with the
latter possibly representing an HLA-restricted subset of PLE.24
Photobiological aspects
Most PLE patients exhibit a sensitivity to sunlight through
window glass while travelling in cars, trains or planes.25 This
observation, along with the lack of protection from UVB-
absorbing sunscreens in the majority of PLE cases, implicate the

role of UVA in triggering the eruption.26 Despite numerous
efforts to define the most effective wavelengths in PLE induc-
tion, the action spectrum of the disease remains elusive. Studies
of the minimal erythemal dose (MED), action spectrum, and
energy doses required to reproduce the lesions have led to
conflicting results because of variations in individual sensitivity,
seasonal variations, geographical differences, and differences
in artificial light sources, light testing protocols and result
interpretations. In general, an abnormal phototest in a photo-
sensitive patient consists of: (i) an abnormal erythemal reaction
to a specific portion of the electromagnetic portion, quantitatively
determined by the MED, and/or (ii) the reproduction of a
typical lesion of the disease following exposure to sunlight or
an artificial light source. Most patients with PLE have a normal
MED value27,28 although a significant number may exhibit a
lowered MED value to UVA or UVB.29,30 Reproduction of PLE
lesions may occur with monochromator phototesting when
using multiples of MED, or with polychromatic UV radiation
(UVR) when exposing larger areas of the skin.12,31,32 The results,
however, are variable and not always reproducible. Nevertheless,
experimental studies using these diagnostic approaches have
suggested that the action spectrum of PLE ranged from UVB to
UVA and into the visible light region, while an additive effect
was also noted with infra-red radiation.33 36 Epstein first
reported that repeated daily exposures, rather than merely a
single exposure, of the same skin site to erythemal doses of UVA
or UVB are necessary to provoke the PLE lesions.37 This con-
cept provided the basis of provocative phototesting or photo-
provocation, a method that is currently considered the most
accepted way of reproducing the lesions of PLE both clinically
and histologically. Depending on the method of photopro-
vocation, PLE lesions can be reproduced in approximately 60
90% of affected subjects, most of whom exhibit sensitivity to
UVA.30,38 40 In one study of 142 subjects in whom induction
was attempted with exposure to increasing doses of UVA
and/or UVB radiation daily for 48 consecutive days, 56% of
the subjects were sensitive to UVA, 17% to UVB and 27% to both
UVA and UVB radiation.38 Similar results were reported by
Holzle et al. who observed positive reactions to UVA in 75%
of their PLE subjects, while 10% were sensitive to UVB, and
15% to both UVA and UVB radiation.30 Lindmaier et al. also
reported UVA sensitivity in the majority of their subjects
(45%), followed by a sensitivity to UVA and UVB (35%) and
UVB alone (20%).39 Other studies, however, have shown a
higher rate of UVB induction in selected individuals.35,41
Epstein42 reported the reproduction of PLE lesions primarily
by UVB wavelengths, and later Miyamoto43 confirmed these
findings by successfully inducing the eruption with UVB in a
high proportion of subjects (57%). Boonstra et al. observed a
pathological reaction to both UVA and UVB in 88% of male
patients and 52% of female patients.44 The remainder of subjects
were UVB sensitive (9% men, 24% women) or UVA sensitive
(3% men, 24% women). Interestingly there was a high propor-
2002 European Academy of Dermatology and Venereology JEADV (2002) 16, 193 206
Polymorphous light eruption 195
tion of male patients with an abnormal reaction to visible light
simultaneously with a sensitivity to UVA and UVB. The role
of visible light in the induction of PLE was not satisfactorily
established in some other studies.30
The lack of a universally accepted, standardized protocol
for photoprovocation in PLE as well as the variations in light
testing methods could explain in part the differences observed
among most photoprovocation studies. However, these dis-
parities could also indicate that PLE is a heterogeneous group of
diseases characterized by different pathogenetic mechanisms or
different cutaneous chromophores. Although the absorbing
chromophore that initiates the PLE eruption is unknown, it is
unlikely that a disease activated by different parts of the UV
spectrum and manifesting with such polymorphism can be
explained based on a single and uniform pathogenetic
mechanism.11 In fact, the induction of PLE lesions by a UVA
sunbed in the non-tanning sacral pressure area suggests that
the photochemical reaction between UVA and the absorbing
molecule occurs in an oxygen-independent fashion.45
The contradictory results regarding the action spectrum of
PLE may be explained also by the inhibiting effect of certain
wavelengths. In an as yet unconfirmed study, the induction
of PLE lesions could not be achieved with broad-band UVA,
although it did occur after the shorter UVA wavelengths were
filtered out.46 It was therefore assumed that longer wave UVA
rays may have a stimulating action, whereas the shorter UVA
bands have an inhibitory effect, presumably interfering with the
generation of some photoallergen or subsequent reactions.
Certain variations in the concentrations of UVA and UVB
in terrestrial sunlight may provide some useful insight in the
pathogenesis of the disease. The higher incidence of PLE in sub-
jects living in temperate areas compared with tropical regions,
may relate to the higher proportion of UVA to UVB rays during
the spring and autumn.8 Furthermore, the higher proportion of
UVB over UVA during the hot summer months may influence
the course of the affection either by reducing inadvertent
UVA exposure or by some UVB-induced alteration of immune
reactivity in PLE subjects.47
Immunological aspects
The clinical and histopathological features of PLE suggest the
presence of a cell-mediated immune response which is induced
by UVR and possibly maintained by impaired immuno-
regulatory mechanisms. The hypothesis regarding involvement
of an immune-mediated mechanism was first proposed in 1942
by Epstein who postulated that a UV-induced neo-antigen may
trigger a delayed-type hypersensitivity reaction.48 Despite
intense efforts over several decades, to date no relevant photo-
allergen has been identified in PLE subjects. Interestingly, a
PLE-like eruption was described as part of a photoallergic
contact dermatitis to Fentichlor.49 This observation suggested
that exposure to this exogenous agent might generate an
196 Stratigos et al.
antigenic photoproduct similar to the UV-induced endogenous
antigen of PLE, or stimulate an immune response that leads to
the recognition of the specific antigeu. Heat shock proteins have
also been implicated as potential epidermal antigens in PLE,
given their importance in autoimmune processes, particularly
in lupus erythematosus.50 In experimentally induced PLE, the
expression of heat shock proteins was found to be elevated in
keratinocytes and endothelial cells of dermal blood vessels from
1 h up to 6 days following UVR.51
Some investigators have also examined the possibility that
PLE involves quantitative or qualitative lymphocyte disturb-
ances that could indicate defective immunoregulatory mech-
anism in affected individuals. Transient decreases as well as
increases in numbers of lymphocytes have been reported.52,53 In
one study, the number of T cells as well as their response to
stimulation with phytohaemagglutinin, concavalin A, and a
purified protein derivative of tuberculin were found to be normal
in subjects with PLE before and after photochemotherapy.54
Furthermore, lymphocytes from PLE subjects were not
activated by exposure to UVR. More recent evidence, however,
has shown that cultured epidermal cells from PLE lesions are
capable of stimulating autologous peripheral blood mono-
nuclear cells after exposure to high doses of UVA or UVB,
suggesting the possibility of immune sensitization against
autologous UV-modified epidermal antigens.55
The immunological nature of PLE was further demonstrated
by the characterization of the inflammatory cells of the PLE
infiltrate. An immunohistochemical study by Norris et al. of
timed skin biopsies from PLE lesions induced by low doses of
solar-simulated radiation demonstrated a prominent perivascular
infiltrate by T cells that was present at 5 h after radiation and
peaked at 72 h.56 CD4+ T cells were more abundant early on,
but, CD8+ T cells dominated at 72 h. Increased numbers of
dermal macrophages as well as dermal and epidermal Langerhans
cells were also seen. These cellular changes resembled those seen
in allergic contact dermatitis and the tuberculin skin reaction,56
and there has also been evidence of changes in the expression of
intracellular adhesion molecules in PLE lesions. Intercellular
adhesion molecule 1 (ICAM-1), a glycoprotein ligand for lym-
phocyte function-associated antigen 1, is strongly expressed
by keratinocytes under the stimulation of interferon-.57 It is
therefore considered a marker of an immune-specific response,
exhibiting increased expression in allergic contact dermatitis
and the tuberculin skin reaction but not in irritant contact
dermatitis or after UVB radiation of normal skin with 2 MED.58
The observation of increased expression of ICAM-1 in lesional
keratinocytes of PLE is probably due to the release of interferon-
by immunologically stimulated lymphocytes in the dermal
infiltrate, which further supports the role of a delayed-type
immune hypersensitivity in this disorder.59
An inherent deficiency in the mechanisms that control the
immune responses of the skin to UV exposure has been also
proposed in the pathogenesis of PLE. After exposure to UVR,
2002 European Academy of Dermatology and Venereology JEADV (2002) 16, 193 206
healthy skin usually demonstrates a dramatic disappearance
of epidermal Langerhans cells, a phenomenon that has been
linked to the UV-induced local suppression of contact hyper-
sensitivity reactions. In patients with PLE, however, Langerhans
cells continued to be present in the epidermis at consider-
able numbers, 24 and 48 h after exposure to 6 MED of UVB
radiation.60 It is possible that this resistance of Langerhans cells
to the immunosuppressive effects of UVB radiation may allow
inadvertent immune reactions to develop after UV exposure,
leading to the clinical manifestations of PLE.
The cytokine profile of the PLE response is largely unknown.
Norris et al. examined the lymphocyte attractant effect of suc-
tion blister fluid samples from PLE subjects before and after
experimental induction of lesions and found an increased activ-
ity of interleukins 6, 8 and possibly 1.61 An earlier study showed
an increased number of apoptotic sunburn cells in PLE subjects
after exposure to 3 and 5 MED compared with normal subjects,
but the role of apoptosis in PLE has not been further explored.62
Biochemical aspects
Abnormalities of arachidonic acid metabolism and prosta-
glandins have been also reported in PLE. Topical application of
indomethacin for 2 h after irradiation inhibited UVB but not
UVA-induced erythema in 13 of 23 subjects with PLE, similar to
the reaction of normal subjects, but caused abnormal augmen-
tation of both UVA and UVB responses in the remaining 10
subjects who presented more severe clinical symptoms and had
shown abnormal erythemal responses to monochromatic UVA
and sometimes UVB radiation.63 This observation may indicate
that PLE comprises two different disease states, the most severe
one associated with abnormal arachidonic acid metabolism in
response to UVR. Dietary supplements of fish oil rich in 3
polyunsaturated fatty acids, that compete with arachidonic acid
and lead to the generation of less active prostanoids, have been
shown to reduce the basal and UVB-generated prostaglandin
levels in skin and increase the threshold for UVA-induced
provocation of PLE.64
Clinical features
The PLE eruption begins typically in the spring or early summer
and tends to improve as the summer progresses. It rarely occurs
in the winter unless the skin is exposed to UVR reflected by the
snow. The period of sun exposure required to induce the
reaction varies from subject to subject, but it usually lasts from
30 min to several hours, and, in cases of long sun abstinence,
several days. The course of the eruption is one of the charac-
teristic features of PLE that distinguishes it from the other
photodermatoses. The skin lesions usually appear after a delay
period of minutes to hours, but not less than 30 min, and, in the
absence of further sun exposure, resolve after 710 days,
and occasionally longer, without scarring or other residual
 Polymorphous light eruption 197

signs.65,66 Discomfort in the form of pruritus or burning may

be experienced by affected subjects prior to the onset of the
eruption. The characteristic sequence of events is pruritus
followed by patchy erythema and finally the onset of distinct
lesions. Occasionally, the rash may present after several successive
days of sun exposure, for example during a beach vacation, giving
the individual the wrong impression of an immediate type
reaction to sunlight.9 Prominent and widespread skin lesions can
be provoked after long periods of seasonal lack of sunshine.67
The predilected sites of PLE lesions are always sun-exposed
areas, particularly those that are normally covered during the
winter, such as the upper chest, the V of the neck, the dorsal
aspects of the arms, and sometimes the shoulders and the lower
legs.5,9,12,13 The face can also be affected and appears to be a
common site of involvement in children. Extension of the rash
to the covered areas of the body is not common, unless there is
light penetration through clothing. Lesions on sun-protected
areas have been reported, however, in patients with long-standing
PLE.68 The lips and eyelids can be affected, and systemic
symptoms, although rare, may occur in the form of headache,
fever, chills and nausea.13 Localized cases of PLE have been
reported to occur solely on vitiliginous skin or on an area of
naevoid telangiectasia.69,70
Despite the name of PLE, the eruption is generally mono-
morphous in any individual subject; different subjects may
fig. 1 Polymorphous light eruption. Confluent papular reaction on the exposed
present different types of lesions. Some individuals may manifest skin of the back of the neck (A) and the extensor forearm (B).
different types of lesions, but a specific morphological pattern
tends to predominate. For example, erythema and swelling can
develop in patients with the papular form of PLE. Several particular, phototoxic reactions, photoallergic reactions, chronic
morphological variants of PLE have been described, including actinic dermatitis, actinic prurigo, photoaggravated atopic der-
the papular, papulovesicular, plaque-type, vesiculobullous, matitis and lupus erythematosus. The other forms are regarded
eczematous, insect bite-like, haemorrhagic and erythema by many investigators as subgroups of the three major PLE
multiforme-like forms (Table 1).13,30,71 Not all of these variants types. The haemorrhagic variant can be considered a subtype
are considered true PLE subgroups. The papular form is the of the papular type, while the vesiculobullous and erythema
most common type (figs 1 and 2), followed by the plaque-type multiforme-like types are regarded as subdivisions of the
(fig. 3) and the papulovesicular type (fig. 4), while there is plaque type and papulovesicular form of PLE, respectively.11
some doubt whether or not the less common eczematous, Other rare variants of PLE have been reported. Dover and
vesiculobullous, insect-bite like, erythema multiforme-like, Hawk reported a pruritic form of PLE with no visible eruption
haemorrhagic and prurigo-like forms are true PLE variants. In (PLE sine eruptione).72 A mild, often delayed-onset clinical
the case of widespread erythema, eczematous appearance and variant of PLE occurring on vacations has been also described
prurigo-like lesions, other entities should be excluded first, in and designated as a benign summer light eruption.73 Another
variant, called juvenile spring eruption, has been reported
Table 1 Clinical types of polymorphous light eruption
in children and youths and is characterized by a self-limiting
eruption of grouped pruritic papules and vesicles on the light-
Papular exposed ear auricles in early spring.74
Papulovesicular During the course of the activity of the disease, some
Plaque-like
patients will show progressive worsening of their symptoms,
Urticarial
Vesiculobullous
while others will exhibit gradual improvement due to skin
Haemorrhagic hardening. This hardening phenomenon occurs after repetitive
Eczematous exposures to UVR during the summer months and results in
Erythema multiforme-like complete tolerance even of intense sunshine towards the end of
Insect bite-like
the summer period.11,13 The frequent sparing of the facial skin,
Prurigo-like
that is continuously exposed to some UVR throughout the year,

2002 European Academy of Dermatology and Venereology JEADV (2002) 16, 193 206
198 Stratigos et al.
fig. 2 Coalescent erythematous papules and plaques of variable sizes on the
chest of a patient with polymorphous light eruption.
the gradual diminution of the rash during the summer in some
patients, and the preventive effect of phototherapy are all
possibly related to the development of immune tolerance
through skin hardening. The main mechanism by which
hardening is achieved involves the suppression of the immune
mechanisms of PLE, although the increased melanization and
thickening of the stratum corneum induced by UVR may also
play a part.25
Histology and immunohistopathology
PLE presents a characteristic, but not pathognomonic, histo-
pathological picture. The basic features of the papular form of
PLE include a moderate to dense perivascular lymphocytic
infiltrate, in the upper and middle dermis, consisting pre-
dominantly of lymphocytes, and, to a lesser degree, neutrophils
and eosinophils (fig. 5).75,76 This inflammatory infiltrate is
often seen in conjunction with dermal oedema and endothelial
cell swelling. Epidermal changes may be present with variable
intensity. They usually consist of vacuolar degeneration of basal
cells, and occasionally spongiosis and exocytosis.
The morphological variants of PLE are characterized by the
same histological pattern, although additional features may be
present and other features may be present. Plaque-type PLE
shows a band-like infiltrate in the upper dermal layers associated
2002 European Academy of Dermatology and Venereology JEADV (2002) 16, 193 206
fig. 3 Erythematous plaques on the face and chest of a female with poly-
morphous light eruption.
with subepidermal oedema, and the erythema multiforme-like
type presents more prominent dermal oedema.11 In the hae-
morrhagic type, there is erythrocyte extravasation in the papillary
dermis. Numerous neutrophils are seen especially in the
papulovesicular form of PLE, whereas the vesiculobullous form
exhibits spongiotic vesicles and severe subepidermal oedema
that leads to blister formation.76 Finally, insect bite-like PLE
demonstrates pronounced epidermal changes with focal necrosis
of keratinocytes.
Immunohistochemically, the dermal infiltrate of PLE is
mainly composed of T-helper cells.77 Macrophages comprise a
smaller part of this infiltrate (less than 12%), whereas the
CD1-positive cells (Langerhans cells and indeterminate
dendritic cells) are increased. The ratio of CD4/CD8 cells in the
dermis depends on the stage of development of the eruption.56
In the early phase, CD4 are more abundant; in later stages,
CD8+ cells predominate. This is in contrast with the lesions
of lupus erythematosus where the majority of dermal
lymphocytes are CD4+ together with some B cells.75
Direct immunofluorescence is usually negative in PLE,
although one study revealed intervascular and focal perivascu-
lar deposition of fibrin as well as C3 and IgM.78 The lupus band

fig. 4 Papulovesicular form of polymorphous light eruption.
fig. 5 Histology of polymorphous light eruption. Moderate perivascular
lymphocytic infiltrate in the dermis with mild subepidermal oedema. No
spongiosis or hydropic degeneration of the basal epidermal layer is noted.
Haematoxylin and eosin, original magnification 63 (Courtesy of Dr Elefteria
Christophidou, Histopathology Unit, A. Sygros Hospital).
test is negative in PLE. Although immunofluorescence could be
used to differentiate between PLE and lupus erythematosus, the
positive results seen in lupus erythematosus are usually found
in persistent lesions of more than 6 weeks duration.79
Diagnostic work-up
The diagnosis of PLE is based mainly on the history and clinical
manifestations. The time course of the eruption and the
morphology of lesions can be helpful, particularly as the
responses of PLE patients to routine phototesting are usually
normal. In cases of diagnostic difficulties, a photoprovocation
2002 European Academy of Dermatology and Venereology JEADV (2002) 16, 193 206
Polymorphous light eruption 199
test may induce the appearance of PLE lesions and a lesional
biopsy will show the characteristic, although not entirely
specific, histological picture of PLE. There are no specific
biochemical abnormalities in PLE and most laboratory exam-
inations are done to exclude other dermatoses. Measurement
of circulating antinuclear antibodies (ANA) and anti-SSA/ Ro
and anti-SSB/La antibody titres are useful in excluding lupus
erythematosus, whereas erythropoietic protoporphyria can
be differentiated from PLE by the elevated levels of blood
protoporphyrin.
Phototesting and photoprovocation
As controversy about the eliciting wavelengths continues,
evidence indicates that the action spectrum of PLE can be in the
UVA and/or UVB wavelength ranges and possibly in the visible
light range. Determination of the MED is usually of limited
diagnostic value, as most PLE patients are reported to have
normal erythemal and pigmentary responses to UVA as well as
to UVB.27,30,38 A number of authors, however, have reported a
decreased MED value either to UVA or UVB radiation.29,30,44
Reproduction of lesions has been reported also by mono-
chromatic or polychromatic phototesting, although with
variable success.12 The repeat phototest or photoprovocation
test remains the most useful procedure for reproducing PLE
lesions, aiming to provoke the pathological reaction by
repeatedly exposing the skin to increasing doses of UVR.
Various protocols have been used but the general principles of
photoprovocation are as follows: two symmetrically located test
areas, preferably on previously involved skin, are exposed daily
for 4 8 days to UVA and UVB radiation, respectively. The
exposure dose is usually one to three times the predetermined
MED in either waveband, depending on the size of the area
exposed.25 If no response is observed, the dose can be increased
by 2040% at each session. The test is considered positive when
a rash typical of the patients PLE variant is elicited. It is
important to note that the eruption may not develop until
23 days after the last exposure. In addition, the site and time
of photoprovocation are critical: exposure of previously
uninvolved skin sites may yield false negative results, whereas
the same may occur when the test is done in late summer
or early fall because of tolerance induction. Therefore, it is
best to do the photoprovocation test before the beginning of
the sunny summer season, preferably in the winter or early
spring.25
In addition to its diagnostic significance, photoprovocation
may also play a part in the prognosis of PLE. This was suggested
by a recent study of affected patients who underwent repetitive
polychromatic phototesting and were followed up on a long-
term basis.80 In patients with negative phototests, the disease
developed at a younger age and had a tendency towards remis-
sion, whereas in patients with positive results the disease tended
to have a more persistent and chronic course.
200 Stratigos et al.
Differential diagnosis
The main differential diagnosis of PLE, both clinically and
histologically, is cutaneous lupus erythematosus, as both
disorders may present with photosensitive eruptions of
erythematous papules on light-exposed areas. The lesions of
subacute and discoid lupus erythematosus, particularly the
tumidus-type of discoid lupus can resemble the plaque-type
PLE, but the prolonged course of lupus erythematosus can help
distinguish between these two entities.81 Lupus erythematosus
lesions emerge 23 weeks after intense sun exposure and
usually persist for several weeks before they gradually resolve.
Other differentiating features of PLE from discoid lupus include
the lack of scalp and ear involvement, the absence of atrophy,
follicular plugging and telangiectasia.82 Also, unlike discoid
lupus erythematosus, PLE tends to show more parakeratosis and
little or infrequent hydropic degeneration and periappendageal
infiltration. The use of direct and indirect immunofluorescent
testing, e.g. ANAs, and anti-SSA/Ro and anti-SSB/La antibodies,
is useful in distinguishing between PLE and the systemic and
subacute forms of lupus erythematosus. In some instances,
however, complete differentiation between lupus erythematosus
and PLE is still difficult, if not impossible. ANAs as well as
anti-SSA/Ro and anti-SSB/La antibodies have been detected in
PLE patients in varying ratios, depending on the substrate used,
but long-term follow-up of these subjects has failed to show
a transition of their disease to lupus erythematosus.83 85 Still,
there is a subgroup of patients with severe photosensitivity,
persistent PLE-like lesions and elevated ANA titres in their
serum that probably represents a forme fruste of lupus eythe-
matosus.86 This observation is supported by a photoprovocation
study of 67 photosensitive patients with confirmed diagnosis of
Table 2 Differential diagnosis of polymorphous light eruption
Diseases
Idiopathic photodermatoses
Solar urticaria
Hydroa vacciniforme
Chronic actinic dermatitis
Porphyrias
Erythropoietic protoporphyria
Other photosensitivity disorders
Lupus erythematosus
Photoallergic dermatitis
Drug photosensitivity
Light-exacerbating photodermatoses (atopic dermatitis)
Other dermatoses
Lymphocytic infiltrate of Jessner
Lymphocytoma cutis
Granuloma faciale
Rosacea
Fixed drug eruption
Insect bites
Erythema multiforme
2002 European Academy of Dermatology and Venereology JEADV (2002) 16, 193 206
lupus erythematosus (discoid, subacute or systemic lupus
erythematosus), showing that in almost half of the subjects
clinical and histological PLE-like lesions were reproduced,
frequently associated with positive serologies for anti-Ro/ La
antibodies.87 These findings indicate that all subjects with PLE
should be screened for lupus erythematosus.
Other diseases that should be considered in the differential
diagnosis of PLE are listed in Table 2. Lymphocytic infiltrate
of Jessner, lymphocytoma cutis and granuloma faciale can be
easily differentiated from the persistent plaque-type of PLE by
microscopic examination. Erythropoietic protoporphyria (EPP)
may occasionally manifest with eczematous or papulovesicular
eruptions, while the pruritic variant of PLE without eruption
can resemble the symptoms of EPP after sun exposure. These
two disorders can be distinguished by measuring the red blood
cell protoporphyrin level and demonstrating increased amounts
of periodic acid-Schiff staining around dermal blood vessels in
histological sections of EPP.88 Solar urticaria may also be con-
fused with PLE by history, but the former tends to have a more
rapid course with the urticarial lesions developing 510 min
after solar exposure and resolving in 12 h.89 Hydroa vaccini-
forme occurs in early childhood and presents with typical clin-
ical manifestations with vesicular lesions that develop after
sun exposure and resolve with varioliform scarring.66 Actinic
prurigo is considered by many investigators to be a variant
of PLE, because of their frequent coexistence as well as some
evidence of a common pathophysiological mechanism.90 Its
distinguishing features from PLE include onset in early child-
hood, a family history of photosensitivity and atopy, the
presence of cheilitis and ocular problems and the occurrence of
the eruption throughout the year with lesions on exposed as
well as covered skin areas.66
Useful diagnostic tools
History, clinical examination, phototesting
History, clinical examination, phototesting
History, clinical examination, phototesting
Red blood cell protoporphyrin, histology
Direct immunofluoresence, serology (ANA, anti-Ro, anti-LA antibodies), histology
Phototesting, photopatch testing
History, phototesting
History, clinical examination
History of sun dependency, clinical examination, histology
History of sun dependency, clinical examination, histology
History of sun dependency, clinical examination, histology
History of sun dependency, clinical examination, histology
History of sun dependency, clinical examination, histology
History of sun dependency, clinical examination, histology
History of sun dependency, clinical examination, histology

Table 3 Treatment of polymorphous light eruption
Topical treatment
Corticosteroids
Sunscreens ( prevention)
Phototherapy
Photochemotherapy (PUVA)
Broad band UVB therapy (290 320 nm)
Narrow band UVB therapy ( 311 nm)
Systemic therapy
Systemic corticosteroids (short courses)
Azathioprine
Antimalarials
Nicotinamide
-carotenes
Antioxidants
When considering the diagnosis of an eczematous form of
PLE other conditions should be excluded, including photo-
exacerbated atopic eczema, allergic contact and photocontact
dermatitis, airborne contact dermatitis and chronic actinic der-
matitis. Light-exacerbating dermatoses, such as atopic dermatitis
and seborrhoeic dermatitis, have a time course similar to that
of PLE but the morphology and distribution of lesions is quite
different and the disease often occurs in the setting of a personal
or family history of atopy. Patch testing can help distinguish
PLE from contact sensitivity, although many PLE patients may
present with a superimposed contact allergy, particularly to
topical preparations or sunscreens. In contrast to PLE, that
tends to avoid skin folds, airborne contact dermatitis is usually
accentuated in folds and flexural areas, such as the creases of
the upper eyelids, the flexures of the wrists, and the antecubital
fossae. The differentiation of PLE from allergic photocontact
dermatitis can pose a difficult diagnostic problem, warranting
extensive phototesting and photopatch testing.
Natural history and prognosis
The long-term prognosis of PLE is not well known. In most
affected subjects the disease has a persistent course with a slow
tendency to amelioration.84,91 The rash tends to recur in the
same skin sites, although its distribution may gradually spread
or recede. Subjects with long-standing PLE have been reported
to develop additional skin lesions on covered skin areas.68
It is not known in what percentage of cases clearing will even-
tually occur nor what the time lapse is for clearing. In one study,
64 of 114 subjects (57%) reported a significant reduction of sun
sensitivity over a period of 7 years, including 12 subjects (11%)
who achieved total clearance of lesions.84 In a subsequent study
of the same cohort with a mean follow-up duration of 32 years
after the onset of PLE, 23 of the 94 (24%) were considered
cured, 48 experienced improvement of symptoms, and 23
(24%) showed equal or worse symptoms.91
Associations with other diseases have been reported in PLE.
A significant number of subjects with PLE develop contact or
2002 European Academy of Dermatology and Venereology JEADV (2002) 16, 193 206
Polymorphous light eruption 201
photocontact allergic reactions, mainly to skin care products
and sunscreens, with positive patch and photopatch testing
demonstrated in up to 75% of cases in some series.44,92 A tend-
ency to develop autoimmune disorders, particularly thyroid
disease, has been reported in PLE subjects. In their series of 94
cases, Hasan et al. reported finding at least one autoimmune
disease in 14 subjects (15%), mostly females, in addition to hypo-
thyroidism or non-toxic goiter of probable autoimmune origin
in eight individuals (8%).91 Autoimmune thyroid disease, rheu-
matoid arthritis and vitiligo were the most frequent autoimmune
associations, but large-scale prospective studies with age- and
sex-matched controls are needed to confirm these observations.
Progression of PLE to other diseases such as lupus erythe-
matosus has been previously discussed. Long-term follow-up
studies of PLE subjects have not shown an increased risk of
transition to lupus erythematosus,84,91 although PLE-like lesions
may precede the development of lupus erythematosus.93 An
overlap of PLE with other photodermatoses, such as actinic
prurigo and chronic actinic dermatitis and possible progression
to the latter, have been reported.90,94
Therapy and prevention
Most cases of PLE are mild, responding well to basic photo-
protective measures, such as sun avoidance and the use of broad-
spectrum sunscreens. In such cases topical corticosteroids
and occasionally oral antihistamines are able to reduce the
inflammation and shorten the duration of the eruption, making
a visit to a dermatologist often unnecessary. Subjects with
moderate to severe PLE, however, experience significant dis-
comfort and life-style restrictions during the summer months
or during vacations in areas with high UV flux. These indi-
viduals with frequent PLE episodes respond well to prophylactic
treatment with phototherapy or photochemotherapy, which
allows most of them to have normal or near-normal lives during
the summer months (Table 3). Subjects with occasional bouts
of disease can be more effectively managed with short courses
of oral corticosteroids, but systemic immunomodulatory
therapy may be necessary in rare cases. Hence, the selection of
the appropriate treatment depends primarily on the frequency,
duration and severity of the disease and the degree of life-style
restriction of affected subjects.95
General measures
Educating affected subjects about the nature of PLE and
providing proper advice on photoprotection are of paramount
importance in the management of this condition. These indi-
viduals should be informed that exposure to UVR induces an
allergic reaction in their skin and that by changing their sun
exposure habits they can avoid pronounced eruptions of PLE. It
is essential therefore that these individuals avoid sun exposure
at times of maximal UV intensity (between 11.00 and 15.00 h).
202 Stratigos et al.
The preferable times of sun exposure during the summer should
be the early morning or evening, unless the subject is particularly
UVA-sensitive. They should also be reminded that a significant
amount of UVR passes through clouds and that sand, snow and
water can reflect UVR and increase its intensity. Sun avoidance
should be combined with the regular use of sunscreens and
protective clothing, such as a wide-brimmed hat and clothing of
lightweight, closely woven fabric or special fabric with a high
sun protection factor. Appropriate sunscreens are the broad-
spectrum sunscreens that also absorb in the UVA region and
have the highest SPF that is cosmetically acceptable to the
patient (usually SPF of 30 or more).
Phototherapy/photochemotherapy
The mechanism by which UV therapy protects against the
development of PLE and other photosensitive eruptions is not
well understood, but it probably involves the induction of
immune tolerance to a putative endogenous UV-modified
antigen in addition to increased skin melanization and
thickening of the stratum corneum.96 The use of phototherapy
simulates the natural-occurring phenomenon of hardening and
aims to induce photoadaptation with small, carefully regulated
doses of UVR without inducing the eruption. A history of
natural hardening is not, however, considered a prerequisite for
a successful desensitization. Treatment is easiest in subjects with
a wide therapeutic window between photoadaptation and
photoprovocation, or who are sensitive to UV wavelengths
different from those used for phototherapy.95 Any of the three
basic phototherapeutic modalities [broad band UVB therapy,
290320 nm; narrow band UVB therapy, 311 nm; photo-
chemotherapy, psoralen + UVA (PUVA)] can be used in the
management of PLE. Selection of the appropriate method is
based on several factors, in particular the age of the subject,
disease severity, response to previous treatments and the type(s)
of phototherapy available.
Several studies have reported the benefits of UVB and PUVA
therapy in PLE. The significant variability of regimens between
centres does not allow for direct comparisons; however, in studies
where broadly equivalent biological doses were used, PUVA
generally achieved better clinical responses and patient satisfaction,
followed by narrow band UVB and broad band UVB.40,9799 In
one controlled study of 122 subjects with PLE treated with
PUVA, 64% achieved total protection and 26% partial protection,
while 10% showed no response.38 Short courses of PUVA
(duration 24 weeks) appear to be just as effective as longer
ones.100 Narrow band UVB phototherapy (TL-01), has become
an increasingly popular method of phototherapy showing an
equal efficacy with PUVA in the prophylaxis of PLE.99 The
advantages of narrow band UVB over PUVA therapy are the
simplicity of delivery and potentially lower carcinogenic risk.
Prophylactic phototherapy usually begins about 1 month
before the expected onset of the eruption. Some subjects experi-
2002 European Academy of Dermatology and Venereology JEADV (2002) 16, 193 206
ence disease exacerbation during treatment, but this is usually
easily controlled with topical or even systemic corticosteroids if
the subject is easily photoprovoked.101 In rare cases, reduction
of UV doses or temporal cessation of treatment may be required.
There is no standard regimen of PUVA or UVB therapy in PLE.
Starting doses vary significantly and some centres use MEDs or
phototoxic doses as guides, although this is not always necessary
as any suberythemal dose can be effective.102 Frequency of treat-
ments and incremental regimens also vary.
We emphasize, furthermore, that suppression of PLE is
temporary, the effect usually lasting for 4 6 weeks. Subjects are
often advised to be cautious in sun exposure to maintain their
photoadaptation. Some individuals may develop long-term
tolerance after several phototherapy sessions, but this appears
to be related to the natural course of the disease rather than the
efficacy of phototherapy.101 It is not clear whether or not
prophylactic phototherapy and photochemotherapy affect the
course of PLE.
Systemic agents
In subjects who experience occasional bouts of the disease, oral
steroids can effectively suppress the PLE reaction; the common
treatment in such cases is a short course of prednisone or pre-
dnisolone administered at the very beginning of the eruption,
or prophylactically prior to a short risk period.103 In two
randomized, placebo-controlled studies, administration of
brief courses of prednisolone from the earliest manifestation of
the eruption were found to be more effective than placebo in
diminishing the PLE reaction,104,105 but short-term therapy
with azathioprine was more beneficial in two severe, refractory
cases of PLE.106,107
Antimalarials have been long considered a useful option in
the treatment of PLE, but recent controlled studies failed to
show convincing and reproducible results. It is possible that
earlier reports of successful treatment may have involved cases of
photosensitive variants of lupus erythematosus misdiagnosed
as PLE. In one study daily doses of 400 mg chloroquine sulphate
showed no significant efficacy compared with placebo.107 On
the other hand, hydroxychloroquine has shown a statistically
significant difference in controlling the symptoms of PLE,
although its therapeutic effect was not pronounced.108
Controversy exists over the therapeutic benefit of nicotina-
mide, which was advocated based on an earlier hypothesis of a
disturbance in tryptophan metabolism in PLE. In an open
study of 42 subjects treated with daily doses of 3 g for 2 weeks,
more than half of the subjects remained asymptomatic despite
sun exposure.19 These results, however, have not been con-
firmed in experimental investigations or controlled studies.
Ortel et al. failed to show an increase in the photoprovocation
threshold of PLE in subjects treated with nicotinamide at
doses similar to those administered by Neumann et al.19,109
Beta-carotene, a well-known quencher of free radicals, has also

been recommended as prophylactic oral treatment for PLE.
Response rates of 30 80% have been reported, but controlled
studies have questioned the efficacy of this agent.110,111 Dietary
supplements of fish oil, rich in 3 polyunsaturated fatty acids,
can increase slightly the threshold for UVA provocation of
lesions, possibly by modifying arachidonic acid and prostaglandin
levels.64 However, the clinical benefits of such supplements
remain uncertain. In addition, several antioxidants, including
ferulic acid, and tocopheryl acetate, can reduce the severity of
provoked PLE, but there is a lack of adequate clinical experience
with these agents.112 Finally, in one unusual case of PLE, where the
subject also had common variable hypogammaglobulinaemia,
treatment with intravenous immunoglobulin led to complete
resolution of PLE.113
References
1 Bateman D. Delineations of Cutaneous Disease. Longman, London,
1817.
2 Hutchinson J. Lectures on Clinical Surgery: on Certain Rare Diseases
of the Skin. Churchill, London, 1879.
3 Rasch C. Om et polymorft (erythematost, vesikulost og
ekzematoidt) lysudslet. Hospitalstid 1900; 43: 478480.
4 Haxthausen H. Studier over Lysdermatirene. II. Undersogesler
over pathologiske Seras Virkning paa fotodermiske Processer.
Hospitalstidende 1918; 577: 19 20.
5 Berg M. Epidemiological studies of the influence of sunlight
on the skin. Photodermatology 1989; 6: 8084.
6 Morison WL, Stern RS. Polymorphous light eruption: a common
reaction uncommonly recognised. Acta Derm Venereol (Stockh)
1982; 62: 237 240.
7 Ros A, Wennersten G. Current aspects of polymorphous light
eruptions in Sweden. Photodermatology 1986; 3: 298302.
8 Pao C, Norris PG, Corbett M et al. Polymorphic light eruption:
prevalence in Australia and England. Br J Dermatol 1994; 130:
62 64.
9 Epstein JH. Polymorphous light eruption. Ann Allergy 1966; 24:
397 405.
10 Everett MA, Crockett W, Lamp JH, Minor D. Light sensitive
eruptions in American Indians. Arch Dermatol 1961; 83: 243 248.
11 Holzle E. Polymorphous light eruption. In: Krutmann J,
Elmets CA, editors. Photoimmunology. Blackwell Science, London,
1995: 167174.
12 Frain-Bell W. The idiopathic photodermatoses. In: Cutaneous
Photobiology. Oxford University Press, Oxford, 1985: 24 59.
13 Jansen CT. The natural history of polymorphous light eruptions.
Arch Dermatol 1979; 115: 165 169.
14 Jansen CT. Heredity of chronic polymorphous light eruptions.
Arch Dermatol 1978; 114: 188 190.
15 Orr PH, Birt AR. Hereditary polymorphous light eruption in
Canadian Inuit. Int J Dermatol 1984; 23: 472475.
16 Sheridan DP, Lane PR, Irving J et al. HLA typing in actinic prurigo.
J Am Acad Dermatol 1990; 22: 1019 1023.
2002 European Academy of Dermatology and Venereology JEADV (2002) 16, 193 206
Polymorphous light eruption 203
17 Lane PR, Sheridan DP, Hogan DJ, Moerland A. HLA typing
in polymorphous light eruption. J Am Acad Dermatol 1991; 24:
570 573.
18 Bonafe JI, Chap H. Lucite polymorphe et perturbation du
metabolisme du tryptophane (voie de la cynurenine). Ann
Dermatol Venereol (Paris) 1980; 107: 89 90.
19 Neumann R, Rappold E, Pohl-Markl H. Treatment of
polymorphous light eruption with nicotinamide: a pilot study.
Br J Dermatol 1988; 118: 669 673.
20 Neumann R. Polymorphous light eruption and oral
contraceptives. Photodermatology 1988; 5: 40 41.
21 Ruzicka T, Przybilla B. Eicosanoid release in polymorphous light
eruption; selective UV-A-induced LTB4 generation by peripheral
blood leucocytes. Skin Pharmacol 1988; 1: 186 191.
22 Millard TP, Bataille V, Snieder H et al. The heritability of
polymorphous light eruption. J Invest Dermatol 2000; 115:
467470.
23 McGregor JM, Grabczynska S, Hawk JLM, Lewis CM. Genetic
modeling of abnormal photosensitivity in families with
polymorphic light eruption and actinic prurigo. J Invest Dermatol
2000; 115: 471476.
24 Menage H, du P, Vaughan RW, et al. HLA-DR4 may determine
expression of actinic prurigo in British patients. J Invest Dermatol
1996; 106: 362 364.
25 Honigsmann H. Polymorphous light eruption. In: Lim HW,
Soter NA, editors. Clinical Photomedicine. Marcel Dekker Inc.,
New York, 1993: 167180.
26 Diffey BL, Farr PM. An evaluation of sunscreens in patients with
broad spectrum photosensitivity. Br J Dermatol 1985; 112: 83 86.
27 Diffey BI, Farr PM. The erythemal response to ultraviolet
radiation in subjects with polymorphic light eruption.
Br J Dermatol 1986; 114: 103 108.
28 Jansen CT. Erythemal and pigmentary phototest reactions
in polymorphous light eruption. Acta Derm Venereol (Stockh)
1979; 59: 499 503.
29 Rottier PB. Testing of the skin for reactions to ultraviolet radiation.
Dermatologica 1963; 127: 260 266.
30 Holzle E, Plewig G, Hofmann C et al. Polymorphic light eruption.
Experimental reproduction of skin lesions. J Am Acad Dermatol
1982; 7: 111125.
31 Verhagen ARHB. Light tests and pathogenetic wavelengths
in chronic polymorphous light dermatosis. Dermatologica 1966;
52: 462 466.
32 Jilson OF, Curwen WL. Phototoxicity, photoallergy, and photoskin
tests. Arch Dermatol 1959; 80: 678 689.
33 Epstein JH. Polymorphous light eruption. Ann Allergy 1966; 24:
397405.
34 Frain-Bell W, Dickson A, Herd J et al. The action spectrum
in polymorphic light eruption. Br J Dermatol 1973; 89: 243 249.
35 Magnus IA. Studies with a monochromator in the common
idiopathic photodermatoses. Br J Dermatol 1964; 76: 245 264.
36 Stevanovic G, Wennersten G. Polymorphous light eruption.
Br J Dermatol 1960; 72: 261270.
204 Stratigos et al.
37 Epstein JH. Polymorphous light eruptions: wavelength
dependency and energy studies. Arch Dermatol 1962; 85: 82 88.
38 Ortel B, Tanew H, Honigsmann H. Polymorphous light eruption.
Action spectrum and photoprotection. J Am Acad Dermatol 1986;
14: 748 753.
39 Lindmaier A, Neumann R. Der PDL Patient. Hauttyp, Hardening
und andere Licht-assoziierte Merkmale. Hautarzt 1991; 42:
430 433.
40 Mastalier U, Kerl H, Wolf P. Clinical, laboratory, phototest and
phototherapy findings in polymorphic light eruptions:
a retrospective study of 133 patients. Eur J Dermatol 1998; 8:
554 559.
41 Cahn MM, Levy EJ, Shafer B et al. Experimentally induced
reactions to ultraviolet light. Polymorphous light eruption and
phototoxicity to drugs. J Invest Dermatol 1959; 32: 355 361.
42 Epstein JH. Polymorphous light eruption. Phototest technique
studies. Arch Dermatol 1962; 85: 502504.
43 Miyamoto C. Polymorphous light eruption: successful
reproduction of skin lesions, including papulovesicular light
eruption, with ultraviolet B. Photodermatology 1989; 6: 69 79.
44 Boonstra HE, van Weelden H, Toonstra J, van Vloten WA.
Polymorphous light eruption: a clinical, photobiologic, and
follow-up study of 110 patients. J Am Acad Dermatol 2000; 42:
199 207.
45 Tegner E, Brudin AM. Polymorphous light eruption
in hypopigmented pressure areas with a UVA sunbed. Acta Derm
Venereol (Stockh) 1986; 66: 446 448.
46 Przybilla B, Galosi A, Heppeler M et al. Polymorphous light
eruption: eliciting and inhibiting wavelengths. Acta Derm Venereol
(Stockh) 1987; 68: 173 176.
47 Baadsgaard O, Cooper KD, Lisby S. et al. Dose response and time
course for induction of T6-DR+ human epidermal antigen-
presenting cells by in vivo ultraviolet A, B, and C irradiation.
J Am Acad Dermatol 1987; 17: 792800.
48 Epstein S. Studies in abnormal human sensitivity to light. IV.
Photoallergic concept of prurigo aestivalis. J Invest Dermatol 1942;
5: 289 295.
49 Norris PG, Hawk JLM, McGibbon DM. Photoallergic contact
dermatitis from Fentichlor. Contact Dermatitis 1988; 18: 318 320.
50 Kaufmann SHE. Heat shock proteins and the immune response.
Immunol Today 1990; 11: 129 136.
51 McFadden JP, Norris PG, Orchard G, Cerio R, Hawk JLM,
Camp RDR. 65 kilodalton heat shock protein expression
in experimentally induced polymorphic light eruption.
Br J Dermatol 1991; 125: 484 (Abstr.).
52 Horkay IJ, Krajczar E, Bodolay E et al. A study on cell-mediated
immunity in polymorphous light eruption. Dermatologica 1983;
166: 75 80.
53 Moncada B, Gonzalez-Amaro R, Baranda ML et al.
Immuno-pathology of polymorphous light eruption. J Am Acad
Dermatol 1984; 10: 970 973.
54 Karvonen J, Viander M, Ilonen J, Jansen CT. PUVA
photosensitization in polymorphous light eruption: evaluation of
2002 European Academy of Dermatology and Venereology JEADV (2002) 16, 193 206
systemic immunological factors. Acta Derm Venereol (Stockh)
1982; 62: 497500.
55 Gonzalez-Amaro R, Baranda L, Salazar-Gonzalez JF et al. Immune
sensitization against epidermal antigens in polymorphous light
eruption. J Am Acad Dermatol 1991; 24: 70 73.
56 Norris PG, Morris J, McGibbon DM et al. Polymorphic light
eruption: an immunopathological study of evolving lesions.
Br J Dermatol 1989; 120: 173 180.
57 Nickoloff B. Role of interferon-? in cutaneous trafficking of
lymphocytes with emphasis on molecular and cellular adhesion
events. Arch Dermatol 1988; 124: 1835 1843.
58 Norris PG, Poston R, Thomas S et al. Expression of endothelial
adhesion molecule-1 (ELAM-1) and intercellular adhesion
molecule-1 (ICAM-1) in the evolution of cutaneous
inflammation: a comparison of ultraviolet B (UVB) erythema and
delayed-type hypersensitivity (DTH). J Invest Dermatol 1991; 96:
763 770.
59 Norris PG, Barker JNWN, Allen MH et al. Adhesion molecule
expression in polymorphic light eruption. J Invest Dermatol 1992;
99: 504 508.
60 Kolgen W, Van Weelden H, Den Hengst S et al. CD11b+ cells and
ultraviolet-B-resistant CD1a+ cells in skin of patients with
polymorphous light eruption. J Invest Dermatol 1999; 113: 410.
61 Norris PG, Bacon K, Bird C et al. The role of interleukins 1, 6, and
8 as lymphocyte attractants in the photodermatoses polymorphic
light eruption and chronic actinic dermatitis. Clin Exp Dermatol
1999; 24: 321326.
62 Katsambas A. Study of the apoptosis phenomenon in patients
suffering from chronic polymorphous light eruption and
comparison of the phenomenon with normal subjects. Subject of
Thesis, University of Athens 1984.
63 Farr PM, Diffey BL. Augmentation of ultraviolet erythema by
indomethacin in actinic prurigo. Evidence of mechanism of
photosensitivity. Photochem Photobiol 1988; 47: 413 417.
64 Rhodes LE, Durham BH, Fraser WD, Friedmann PS. Dietary fish
oil reduces basal and ultraviolet B-generated PGE2 levels in skin
and increases the threshold to photoprovocation of polymorphic
light eruption. J Invest Dermatol 1995; 105: 532 535.
65 Holzle E, Plewig G, Hofmann C, Roser-Maass E. Polymorphous
light eruption. J Invest Dermatol 1987; 88: 325 385.
66 Norris PG, Hawk JLM. The idiopathic photodermatoses:
Polymorphic light eruption, actinic prurigo, and hydroa
vacciniforme. In: Hawk JLM, editor. Photodermatology.
Arnold, London, 1999: 103 111.
67 Ippen H. Photodermatitis multiformis acuta. Dermatol
Monatsschr 1980; 166: 145 150.
68 Lindmaier A, Neumann R. Der PDL-Patient. Hautarzt 1991; 42:
430 433.
69 Downs AM, Lear JT, Dunnill MG. Polymorphic light eruption
limited to areas of vitiligo. Clin Exp Dermatol 1999; 24: 321 326.
70 Creamer D, Clement M, McGregor JM, Hawk JL. Polymorphic
light eruption occurring solely on an area of nevoid telangiectasia.
Clin Exp Dermatol 1999; 24: 202 203.

71 Elpern DJ, Morison WL, Hood AF. Papulovesicular light eruption.
A defined subset of polymorphous light eruption. Arch Dermatol
1985; 121: 1286 1288.
72 Dover JS, Hawk JLM. Polymorphic light eruption sine eruptione.
Br J Dermatol 1988; 118: 73 76.
73 Leonard F, Morel M, Kalis B et al. Psoralen plus ultraviolet A
in the prophylactic treatment of benign summer light eruption.
Photodermatol Photoimmunol Photomed 1991; 8: 9598.
74 Berth-Jones J, Hutchinson PE, Burns D et al. Juvenile spring
eruption of the ears. A re-examination. Br J Dermatol 1989; 121
(Suppl. 34): 51.
75 Lever WF, Schaumburg-Lever G. Polymorphous light eruption.
In: Lever WF, Schaumburg-Lever G, editors, Histopathology of
the Skin, 7th edn. JB Lippincott, Philadelphia, 1990: 232233.
76 Hood AF, Elpern DJ, Morison EL. Histopathologic findings in
papulovesicular light eruption. J Cutan Pathol 1986; 13: 1321.
77 Muhlbauer JE, Bhan AK, Harrist TJ et al. Papular polymorphic
eruption: an immunoperoxidase study using monoclonal
antibodies. Br J Dermatol 1983; 108: 153162.
78 Muhlbauer JE, Mihm MC, Harrist TJ. Papular polymorphous light
eruption. Fibrin, complement, and immunoglobulin deposition.
Arch Dermatol 1984; 120: 866 868.
79 Cripps DJ, Rankin J. Action spectra of lupus erythematosus and
experimental immunofluorescence. Arch Dermatol 1973; 107:
563 567.
80 Leroy D, Dompmartin A, Faguer K et al. Polychromatic phototest
as a prognostic tool for polymorphic light eruption. Photodermatol
Photoimmunol Photomed 2000; 16(4): 161166.
81 Lehmann P, Holzle E, Kind P, Goerz G, Plewig G. Experimental
reproduction of skin lesions in lupus erythematosus by UVA and
UVB radiation. J Am Acad Dermatol 1990; 22: 181187.
82 Epstein JH. Polymorphous light eruption. Dermatol Clin 1986; 4:
243 251.
83 Kiss M, Husz S, Dobozy A. The occurrence of antinuclear,
anti-SSA/Ro and anti-SSB/La antibodies in patients with
polymorphous light eruption. Acta Derm Venereol (Stockh) 1991;
71: 341 343.
84 Jansen CT, Darvonen J. Polymorphous light eruption. A seven year
follow up evaluation of 114 patients. Arch Dermatol 1984; 120: 862.
85 Murphy GM, Hawk JL. The prevalence of antinuclear antibodies
in patients with apparent polymorphic light eruption. Br J
Dermatol 1991; 125: 448 451.
86 Petzelbauer P, Binder M, Nikolakis P et al. Severe sun sensitivity
and the presence of antinuclear antibodies in patients
with polymorphous light eruption-like lesions. A forme fruste of
photosensitive lupus erythematosus? J Am Acad Dermatol 1992;
26: 68 74.
87 Hasan T, Nyberg F, Stephansson E et al. Photosensitivity
in lupus erythematosus, UV photoprovocation results compared
with history of photosensitivity and clinical findings. Br J Dermatol
1997; 136: 699 705.
88 Murphy GM. The cutaneous porphyrias: a review. The British
Photodermatology Group. Br J Dermatol 1999; 140: 573581.
2002 European Academy of Dermatology and Venereology JEADV (2002) 16, 193 206
Polymorphous light eruption 205
89 Uetsu N, Miyauchi-Hashimoto H, Okamoto H, Horio T.
The clinical and photophotobiological characteristics of solar
urticaria in 40 patients. Br J Dermatol 2000; 142: 32 38.
90 Grabczynska SA, McGregor JM, Kondeatis E et al. Actinic prurigo
and polymorphic light eruption: common pathogenesis and
the importance of HLA-DR4/DRB1*0407. Br J Dermatol 1999;
140: 232 236.
91 Hasan T, Ranki A, Jansen CT, Karvonen J. Disease associations
in polymorphous light eruption. A long term follow-up study of
94 patients. Arch Dermatol 1998; 134: 10811085.
92 Bilsland D, Ferguson J. Contact allergy to sunscreen chemicals
in photosensitivity dermatitis/actinic reticuloid syndrome
(PD/AR) and polymorphic light eruption (PLE). Contact
Dermatitis 1993; 29: 70 73.
93 Nyberg F, Hasan T, Puska P et al. Occurrence of polymorphous
light eruption in lupus erythematosus. Br J Dermatol 1997; 136:
217221.
94 Gudmundsen KJ, Murphy GM, OSullivan D et al. Polymorphic
light eruption with contact and photocontact dermatitis.
Br J Dermatol 1991; 124: 379 382.
95 Millard TP, Hawk JLM, Travis LB, McGregor JM. Treatment of
polymorphic light eruption. J Dermatol Treat 2000; 11: 195 199.
96 Honigsmann H. Psoralen photochemotherapy mechanisms,
drugs, toxicity. Curr Probl Dermatol 1986; 15: 52 66.
97 Murphy GM, Hawk JLM, Magnus IA. Prophylactic PUVA and
UVB therapy in polymorphous light eruption: a controlled trial.
Br J Dermatol 1987; 116: 531538.
98 Addo HA, Sharma SC. UVB phototherapy and
photochemotherapy (PUVA) in the treatment of polymorphic
light eruption and solar urticaria. Br J Dermatol 1987; 116:
539 547.
99 Bilsland D, George SA, Gibbs NK et al. A comparison of narrow
band phototherapy (TL-01) and photochemotherapy (PUVA)
in the management of polymorphic light eruption. Br J Dermatol
1993; 129: 708 712.
100 Menage HP, Norris PG, Cheong WK. Short course of PUVA
therapy is effective in polymorphic light eruption. Br J Dermatol
1992; 127 (Suppl. 40): 32.
101 Man I, Dawe RS, Ferguson J. Artificial hardening for polymorphic
light eruption: practical points from ten years experience.
Photodermatol Photoimmunol Photomed 1999; 15: 96 99.
102 British Photodermatology Group. Guidelines for PUVA.
Br J Dermatol 1994; 130: 246 255.
103 Molin L, Volden G. Treatment of polymorphous light eruption
with PUVA and prednisolone. Photodermatology 1987; 4:
107108.
104 Creamer D, Patel D, Bellamy G et al. Assessment of the efficacy
of a short course of oral prednisolone in the treatment of
polymorphous light eruption. Br J Dermatol 1999; 141 (Suppl. 55):
74.
105 Patel DC, Bellaney GJ, Seed PT et al. Efficacy of short-course oral
prednisolone in polymorphous light eruption: a randomized
controlled trial. Br J Dermatol 2000; 143: 828 831.
206 Stratigos et al.

106 Norris PG, Hawk JLM. Successful treatment of severe
polymorphous eruption with azathioprine. Arch Dermatol 1989;
125: 13771379.
107 Corbett MF, Hawk JLM, Herxheimer A, Magnus IA. Controlled
therapeutic trials in polymorphic light eruption. Br J Dermatol
1982; 107: 571 581.
108 Murphy GM, Hawk JL, Magnus IA. Hydroxychloroquine
in polymorphic light eruption: a controlled trial with drug and
visual sensitivity monitoring. Br J Dermatol 1987; 116:
379 386.
109 Ortel B, Wechdorn D, Tanew A, Honigsmann H. Effect of
nicotinamide on the phototest reaction in polymorphous light
eruption. Br J Dermatol 1988; 118: 669673.
110 Swanbeck G, Wennersten G. Treatment of polymorphous light
eruptions with beta-carotene. Acta Derm Venereol 1972; 52:
462 466.
111 Jancen CT. Oral carotenoid treatment in polymorphous light
eruption: a cross over comparison with oxychloroquine and
placebo. Photodermatology 1985; 2: 166 169.
112 Hadshiew I, Stab F, Untiedt S et al. Effects of topically applied
antioxidants in experimentally provoked polymorphous light
eruption. Dermatology 1997; 195: 362 368.
113 Creamer D, McGregor JM, Hawk JL. Polymorphic light eruption
occurring in common variable hypogammaglobulinaemia, and
resolving with intravenous immunoglobulin therapy. Clin Exp
Dermatol 1999; 24: 273 274.

Appendix: multiple choice questions 5 The best method to confirm the diagnosis of PLE is:
A Phototesting to determine the MED
Questions 1 6 B Photoprovocation
In each if the following questions choose ONE correct answer. C Photopatch testing
D Patch testing
1 Which of the following clinical types of PLE is the most
E Direct immunofluorescence
common?
A Vesiculobullous type 6 What is the main mechanism by which PUVA therapy pre-
B Papular type vents PLE?
C Eczematous type A Induction of tanning
D Erythema-multiforme like B Induction of skin hyperplasia
E Urticarial C Induction of immune tolerance
D Inhibition of keratinocyte proliferation
2 What is the predominant cell in the dermal inflammatory
E Inhibition of mast cell degranulation
infiltrate in PLE?
A Eosinophil Questions 710
B Mast cell Indicate which of the following questions are true or false.
C Lymphocyte
7 The onset of PLE occurs usually 515 min after sun exposure.
D Fibroblast
A True
E Basophil
B False
3 Which portion of the solar spectrum is most commonly
8 Most patients with PLE have an abnormal MED value.
responsible for the induction of PLE lesions?
A True
A UVA (320 400 nm)
B False
B UVB (290 320 nm)
C UVC (200 290 nm) 9 The detection of anti-Ro/La antibodies in PLE subjects helps
D Visible light to distinguish this disease from discoid lupus erythematosus.
E Infrared light A True
B False
4 Which of the following diseases is frequently seen in PLE
patients? 10 PLE has a persistent course with a tendency to improvement
A Asthma in most affected subjects.
B Iron deficiency anaemia A True
C Cholelithiasis B False
D Depression
The correct answers are 1B, 2C, 3A, 4E, 5B, 6C, 7B, 8B, 9B, 10A.
E Autoimmune thyroid disorder

2002 European Academy of Dermatology and Venereology JEADV (2002) 16, 193 206