Chapter 91

Abnormal Responses
to Ultraviolet
Radiation: Idiopathic,
Probably Immunologic,
and Photoexacerbated

T-cell lymphoma, and very abnormal responses to

the UVB, UVA, and occasionally visible wavelengths
were also seen. Furthermore, although the authors
noted a resemblance to persistent light reactivity,
results of photopatch testing to all common photoallergens were negative. Similar but purely eczematous variants of the condition, also without overt
preceding photoallergy, were then described, with
action spectra limited to the UVB range (photosensitive eczema)69 or UVB and UVA ranges (photosensitivity dermatitis).70

Travis W. Vandergriff
& Paul R. Bergstresser

In 1979, Hawk and Magnus71 proposed that actinic

reticuloid, photosensitive eczema, and photosensitivity dermatitis were part of the same syndrome,
which they termed chronic actinic dermatitis (CAD),
following reports that photosensitive eczema could
progress to actinic reticuloid and vice versa.72 This
analysis was further supported by the occasional
association of the clinicopathologic features of
actinic reticuloid with just UVB photosensitivity,71 a
final theoretical variant of CAD not previously described. Furthermore, in view of the marked clinical,
histologic, and photobiologic similarities between
persistent light reaction and CAD, it was later decided that these two entities were also the same.

ACQUIRED IDIOPATHIC,
PROBABLY IMMUNOLOGIC
PHOTODERMATOSES
Chronic Actinic Dermatitis
HISTORICAL ASPECTS. (see Table 91-2).
Persistent light reactivity was first noted in 1933 in
a patient who was given intravenous trypaflavine.63
It was not reported again until 1960 when there
was an outbreak of persistent photosensitivity that
followed photocontact dermatitis to tetrachlorosalicylanilide, an antibacterial agent used at that time
in soaps and toiletries.64 This condition was later
named persistent light reaction,65 which referred
to a severe, persistent photosensitivity of not just
exposed but also unexposed skin that developed
rarely after photoallergic contact sensitization. The
halogenated salicylanilides were implicated as were
other substances such as musk ambrette66 and
quinoxaline dioxide, and usually in elderly males.67
An initial photocontact dermatitis persisted in rare
instances despite complete avoidance of further
chemical exposure, and the wavelengths inducing
the disorder changed from UVA alone to include
UVB radiation or even UVB radiation exclusively.
Even visible light was implicated, albeit rarely.
In 1969, Ive et al68 introduced the term actinic
reticuloid to describe a similar but more severe
disorder not associated with prior photocontact
dermatitis. The condition again affected elderly
males primarily, although it was characterized by
more infiltrated eczematous plaques, mainly on
exposed sites. The histopathologic features in these
patients tended to resemble those of cutaneous

PHOTOEXACERBATED
DERMATOSES
Acne
(See Chapter 80)
Acne aestivalis, first described by Hjorth et al,123
was characterized by pruritic, 1- to 3-mm, pink or
pale, dome-shaped papules that occurred after
sun exposure, usually on the face, neck, or trunk.
Nieboer124 described the disorder as actinic superficial folliculitis, a predominantly follicular, pustular
rash occurring several hours after sun exposure
and affecting the upper trunk and arms. Verbov125
and Veysey126 described additional patients with
overlapping features of both acne aestivalis and
actinic superficial folliculitis, suggesting the unifying term actinic folliculitis. The condition appears to
be a form of UVR-exacerbated acne, for which high
protection-factor sunscreens, standard acne treatments, including topical retinoic acid, and topical
and systemic antibiotics have not generally been
helpful, although oral isotretinoin appears to be
effective.122

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116 Chapter91: AbnormalResponsestoUltravioletRadiation:Idiopathic,ProbablyImmunologic,andPhotoexacerbated

Darier Disease
(See Chapter 51)
Exacerbation of the eruption after sun exposure is
also well recognized, but mechanisms for this effect
are not known.

Disseminated Superficial Actinic

Porokeratosis
(See Chapter 52)
Disseminated superficial actinic porokeratosis
most commonly affects fair-skinned individuals in
sunny climates. Susceptibility may be inherited as
an autosomal dominant trait. Lesions generally first
appear in the third or fourth decades, gradually
increasing in frequency with age. Each begins as a
conical, horny papule extending to form a brown
red circinate lesion with a peripheral sharp, slightly
raised, keratotic rim and often also with a mildly
atrophic, erythematous, or hyperpigmented central
area. Disseminated superficial actinic porokeratosis
may be induced or exacerbated by sun exposure or
by immunosuppression,127 which suggests that exacerbation by sunlight in part reflects UVR-induced
impairment of local cutaneous immunity.128
Several authors have also reported the induction of new lesions or exacerbation of preexisting
ones after exposure to artificial UVR sources.129
New lesions have been triggered during long-term
phototherapy for psoriasis,130132 and the disorder
has also been aggravated by exposure in suntan
parlors. Further, in a study of potentially provocative wavelengths, UVB plus UVA was more effective
in inducing new lesions or exacerbating preexisting
ones than either wavelength alone.133

Herpes Simplex
(See Chapter 193)
It is well established that many patients experience herpes simplex eruptions after sun exposure, particularly after skiing or other recreational
exposure. The mechanism for such viral activation
by UVR may be related to localized UVR-induced
immunosuppression.

Lichen Planus Actinicus

(See Chapter 26)

In addition to being able to trigger lichen planus

as a Koebner reaction to sunburn injury, UVR in suberythemogenic doses may also induce a lichenoid
reaction known as lichen planus actinicus.134 The
condition most commonly affects darker skinned
persons, particularly those from the Middle East,
but affected individuals have also been reported in
India, Europe, and the United States. Several clinical
patterns occur, the most common consisting of
annular, hyperpigmented plaques predominantly
on the face and dorsa of the hands. Others include
pigmented melasma-like patches of the face and
neck and skin-colored, closely aggregated, pinhead papules, particularly of the face and dorsa of
the hands.134,135 The peak age of onset is the third
decade, and the lesions are generally only mildly
pruritic, developing mainly on exposed sites during
spring and summer and improving or remitting in
winter. Experimental reproduction of lesions was
successful with UVB but not with UVA radiation in
one patient.136 On the other hand, phototherapy
has been used successfully to treat conventional
lichen planus.137,138

Eczema
(See Chapters 15 and 16)
Patients with atopic or seborrheic eczema occasionally report mild to moderate, and at times
severe, exacerbation of the condition after sun
exposure. However, the majority of patients with
atopic or seborrheic eczema benefit from both
sunlight and artificial UV irradiation.

Lupus Erythematosus
(See Chapter 155)
Photosensitivity has been reported in lupus
erythematosus (LE)139 and dermatomyositis.140
However, only in lupus is the correlation between
sunlight exposure and development of skin lesions
well established. Therefore, photosensitivity resulting in a specific skin eruption has been added to
the American Rheumatological Association list of
criteria for the diagnosis of systemic lupus. The estimated prevalence of photosensitivity in the disease
ranges from 30%70% in the white population. It is
important to recognize that patients with different
subtypes of lupus show different degrees of photosensitivity. Chronic cutaneous lupus with typical
discoid lesions has not generally been believed to
be photoexacerbated, although photoexacerba-

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Chapter91: AbnormalResponsestoUltravioletRadiation:Idiopathic,ProbablyImmunologic,andPhotoexacerbated 117

tion has occasionally been reported. However, the

discoid lesions do occur primarily on sun-exposed
areas such as the face, scalp, and ears, and some
patients with discoid lupus progress to the systemic
form. Whether such progression occurs only in
originally photosensitive discoid lupus patients, or
whether all discoid lupus patients are photosensitive, remains unclear. The typical systemic lupus
butterfly rash may also appear suddenly after
sun exposure, whereas widespread lesions may
also develop on other exposed skin areas during
systemic exacerbations. However, the lupus subset
associated most strikingly with photosensitivity is
the subacute cutaneous form. Approximately 50%
of these patients fulfill the American Rheumatological Association criteria for systemic lupus, but their
systemic manifestations tend to be milder than in
those with classical systemic lupus.141,142
The action spectrum for the induction of lupus
skin lesions includes both the UVB and the UVA
wavebands, and there are also scattered single
reports of photoexacerbation during photochemotherapy28 for psoriasis. Lehmann et al143 investigated the artificial UVR-induced reproduction
of lupus lesions in 128 patients. They produced
lesions on exposure to UVA and UVB radiation in
53% of patients, UVA radiation alone in 14%, and
UVB radiation alone in 33%. Because exacerbations
can be provoked by both UVA and UVB irradiation,
appropriate broad-spectrum sun protection should
be recommended for patients with all forms of
lupus.141

Pellagra
(See Chapter 130)
Pellagra is characterized clinically by skin abnormalities, gastroenteritis, and encephalopathy
(dermatitis, diarrhea, and dementia). The cutaneous features are often sunlight induced, appearing in the spring and summer and improving in
winter. Pruritus and erythema occur initially on
exposed areas, followed by vesicles, bullae, and
largely symmetric, chronic, scaly, hyperpigmented,
thickened sclerotic papules and plaques. Dusky
erythema and powdery scaling of the nose, a
scaling collarette around the neck with sternal
extension (Casals necklace), glossitis, and mucous
membrane ulcerations are also typical. The inducing wavelengths are unknown, because no lesions
or other abnormalities have been demonstrated
after artificial irradiation, but it seems likely that
a decreased availability of nicotinamide adenine

dinucleotide phosphate and reduced nicotinamide

adenine dinucleotide phosphate resulting from the
niacin deficiency may prevent the oxidationreduction reactions necessary for the normal repair
of UVR-induced epidermal damage, such that it is
very likely that UVB irradiation is responsible for the
induction of pellagra lesions.

Pemphigus
(See Chapter 54)
Pemphigus foliaceus and pemphigus erythematosus may be exacerbated or induced by sun exposure, and lesions with the characteristic histologic
and immunofluorescent features have been produced in patients with both variants with artificial
UVR exposure. Pemphigus vulgaris is not associated
clinically with photosensitivity.

Bullous Pemphigoid
(See Chapter 56)
Precipitating factors appear to be rare in the
development of bullous pemphigoid, but a few
patients have developed lesions after UVB radiation
exposure, and more after psoralen photochemotherapy (PUVA) given for unrelated diseases.144

Psoriasis
(See Chapter 18)
Some patients with psoriasis report exacerbation
of their disease after sun exposure and particularly
after sunburn, and several groups have attempted
to estimate the prevalence of such photosensitivity with surveys and questionnaires. Lomholt145
reported that 24% of female and 14% of male
patients were aware of a similar phenomenon; and
Farber et al146 noted that 15% experienced a deterioration in their condition after sun exposure. Ros
et al, however, after administering questionnaires
to or conducting telephone interviews with 2,000
patients in Stockholm, considered the prevalence
of photosensitivity to be only 5.5%.147 In this study,
43% of the light-sensitive psoriasis patients were
considered to have a history of PMLE leading to the
psoriatic exacerbation, and they were also more
likely to have skin phototype I, psoriasis of the
backs of their hands, a family history of psoriasis,
and an advanced age.

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