photoaggravated pityriasis rubra pilaris

3
Brief communication Photoaggravated pityriasis rubra pilaris G. Evangelou 1 , S. R. Murdoch 2 , I. Palamaras 1 , L. E. Rhodes 1 1 Photobiology Unit, Dermatological Sciences, University of Manchester, Hope Hospital, Manchester, UK, and 2 The 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/cm 2 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. Discussion We 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: 272–274 Blackwell Munksgaard Copyright r Blackwell Munksgaard 2005 272

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Page 1: Photoaggravated pityriasis rubra pilaris

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

Page 2: Photoaggravated pityriasis rubra pilaris

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

Page 3: Photoaggravated 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]

274

Evangelou et al.