photoaggravated pityriasis rubra pilaris
TRANSCRIPT
Brief communication
Photoaggravated pityriasis rubra pilaris
G. Evangelou1, S. R. Murdoch2, I. Palamaras1, L. E. Rhodes1
1Photobiology Unit, Dermatological Sciences, University of Manchester, Hope Hospital, Manchester, UK, and 2The Princess Royal Hospital,
Telford, UK
Pityriasis rubra pilaris (PRP) is a rare papulosqua-
mous condition with an estimated incidence of one in
35 000 to one in 50 000. Psoralen and ultraviolet A
(UVA) therapy has been used in its treatment but some
patients are reported to be clinically photosensitive.
We describe the photoinvestigation of a patient with
PRP in whom sensitivity to broadband UVA was
demonstrated.
Key words: photoaggravation; pityriasis rubra pilaris;
ultraviolet – A.
A 71-year-old man was referred to the photobiol-
ogy unit with a history of an intensely pruritic
rash following a summer holiday in Ireland 1 year
previously. The rash affected principally the sun-
exposed areas, i.e. his arms, face, neck and upper
chest, but continued over the winter months. The
patient was aware that sunlight aggravated his condi-
tion, becoming more prominent 8–12 h following ex-
posure. It could be provoked by light transmitted
through window glass. There was no family history
of photosensitivity, atopy or other skin disorders. He
was of sun-reactive skin type II. Topical steroids had
not controlled his problem. On examination there was
a widespread well-demarcated orange–red scaly rash
with islands of normal skin (Fig. 1) affecting the face,
V of chest, trunk, arms and legs, and with thicker scaly
plaques in the scalp. The rash extended over the back
and down to the buttocks but the eruption was
markedly worse on sun-exposed areas. There was
also nail involvement with subungual hyperkeratosis,
prominent ectropion and mild plantar hyperkeratosis.
Monochromated light testing (Oriel Ltd., Surrey,
UK) to narrow bands of ultraviolet B (UVB) (300 �5 nm), ultraviolet A (UVA) (320 � 10, 330 � 10,
350 � 20, 370 � 20 nm) and visible light (400 � 20,
500 � 20, 600 � 20 nm) showed normal erythemal
thresholds. The patient received three consecutive
daily challenges of 20 J/cm2 of broadband UVA to
previously uninvolved skin on his left forearm. This
provoked an erythema with scaly topped papules very
similar to his coexisting eruption (Fig. 2a). The same
provocation challenge was performed again 4 months
later, producing a rash of the same morphology.
Biopsy of his naturally occurring rash revealed mild
hyperkeratosis with prominence of the granular layer,
acanthosis and a mild perivascular lymphocytic infil-
trate, while biopsy from the UVA-induced rash was
similar, showing hyperkeratosis, hypergranulosis and
acanthosis (Fig. 2b). Although non-specific, the his-
tological features of both biopsies were consistent
with the diagnosis of pityriasis rubra pilaris (PRP).
Full blood count, ESR, and routine biochemistry were
all normal. Direct immunofluorescence, porphyrin
and autoantibody screens were negative.
DiscussionWe conclude that this patient’s underlying disorder is
PRP with associated UVA photosensitivity. He ex-
hibited features of type I PRP (classical adult form),
with an extensive erythema with areas of normal skin,
spreading in a cephalo-caudal direction (1). Suppor-
tive diagnostic features were follicular plugging with
perifollicular erythema, plantar and scalp involvement
and ectropion. The involvement of exposed more than
unexposed skin and worsening in the summer months,
as well as the provocation by artificial UVA irradia-
tion, is in keeping with a photoaggravated rash. A
review by Griffiths (1) reported that improvement of
PRP could occur in the summer in some patients,
while sunlight could also aggravate the condition.
Davidson et al. (2) reported in a series of 57 cases of
PRP, that 15 patients (26%) showed exacerbation in
the summer months. One previous patient with PRP
Photodermatol Photoimmunol Photomed 2005; 21: 272–274Blackwell Munksgaard
CopyrightrBlackwellMunksgaard 2005
272
has been reported to have undergone photoinvestiga-
tion (3). In this 59-year-old man, repeated broadband
UVB challenge (three MED on 3 consecutive days)
precipitated erythema and scaling at the site after 10
days. In contrast our patient exhibited successful
provocation of the condition with broadband UVA,
and the rash was seen the day following three con-
secutive daily challenges.
Psoralen and UVA therapy (PUVA) has been used
for the treatment of PRP but has shown variable
clinical results. Some patients have not shown any
improvement in their condition, while in others the use
of PUVA or UVA1 alone or combined with retinoids
has been reported to be beneficial (4, 5). The current
report illustrates that PRP can be accompanied by
marked photosensitivity to UVA and we suggest that
this possibility should be taken into account prior to
consideration of treatment with phototherapy. Our
Fig. 1. (a) A widespread erythematous eruption morepronounced on exposed sites with islands of normalskin. (b) Ectropion and (c) subungual hyperkeratosisare evident.
Fig. 2. This shows (a) a scaly papular rash followingprovocation with broadband ultraviolet-A (UVA) inpreviously uninvolved skin and (b) histology of theprovoked rash, revealing hyperkeratosis (orthokera-tosis), hypergranulosis and acanthosis.
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Pityriasis rubra pilaris
patient’s eruption has been successfully controlled with
the use of systemic retinoids (Acitretin) and photo-
protective measures.
References1. Griffiths WAD. Pityriasis rubra pilaris. An historical approach.
II. Clinical features. Clin Exp Dermatol 1976; 1: 37–50.
2. Davidson CL, Winkelman RK, Kierland RR. Pityriasis rubrapilaris: a follow up study of 57 patients. Arch Dermatol 1969;
100: 175–178.
3. Marguery MC, Durand-Malgoures C, Bayle-Lebey P, Dupin P,Bazex J. Photosensitive and phototriggered pityriasis rubra pilaris.
Photodermatol Photoimmunol Photomed 1994; 10: 42–45.
4. Neess CM, Hinrichs R, Dissemond J, et al. Treatment of
pruritus by capsaicin in a patient with pityriasis rubra pilarisreceiving RE-PUVA therapy. Clin Exp Dermatol 2000; 25:
209–211.
5. Herbst M, Vogelbruch A, Ehinis P, Kiehl A. Combined ultra-
violet A1 radiation and acitretin therapy as a treatment optionfor pityriasis rubra pilaris. Br J Dermatol 2000; 142: 574.
Accepted for publication 29 June 2005
Corresponding author:
L. E. Rhodes
Photobiology Unit
Dermatological Sciences
University of Manchester School of Medicine
Hope Hospital, Manchester M6 8HD
UK
Tel: 10161 206 1150
Fax: 10161 206 1156
e-mail: [email protected]
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Evangelou et al.