The photosensitivity dermatitis and actinic reticuloid syndrome(chronic actinic dermatitis) occurring in seven young atopicdermatitis patients

S.C.RUSSELL, R.S.DAWE, P.COLLINS, I.MAN AND J.FERGUSONPhotodermatology Unit, Department of Dermatology, Ninewells Hospital and Medical School, Dundee DD1 9SY, U.K.

Accepted for publication 17 October 1997

Summary Seven young patients with atopic dermatitis (AD) who presented with a marked photoexposed sitedermatitis have been investigated in detail. The results of phototesting, patch testing and otherinvestigations were compatible with the diagnosis of photosensitivity dermatitis/actinic reticuloidsyndrome (PD/AR) (chronic actinic dermatitis). It is known that AD patients may have photoag-gravation of their dermatitis or exacerbation secondary to a photodermatosis, such as polymorphiclight eruption, actinic prurigo or drug-induced phototoxicity. The patients we describe, however,appear to be an uncommon AD subgroup affected by PD/AR. We recommend that all AD patientswho have a history of sunlight-induced exacerbation or marked intolerance of PUVA or ultraviolet Bphototherapy should have phototesting and patch testing conducted.

The photosensitivity dermatitis/actinic reticuloid syn-drome (PD/AR) (chronic actinic dermatitis, CAD) is anidiopathic photodermatosis that typically affects elderlymales.1 The condition is usually characterized by aperennial, chronic dermatitis, maximal on photo-exposed sites, with less severe covered site involvement.Phototesting, the essential investigation to establish thediagnosis, reveals photosensitivity to both ultraviolet(UV) B (290–320 nm) and UVA wavelengths (320–400 nm) in 95% of patients. In addition, sensitivity tovisible wavelengths (>400 nm) is seen in 50% of cases.2

The morphology and histopathology of the phototestsite is of dermatitis, rather than the sunburn UVRresponse seen in normal subjects. Patch testing revealsmultiple contact allergies in 70% of patients, chiefly toconstituents of the European Standard Series,3 fra-grances,4 absorbent sunscreens5 and the Compositaeoleoresins.6

Approximately half of affected patients are unawareof the role of sunlight at presentation; this is usually dueto visible/UVA wavelength induction which may occureven on cloudy days. In such cases it is important tomake the diagnosis in order to provide appropriatemanagement. Five hundred and five patients with PD/AR have been assessed in the Photobiology Unit inDundee during the period 1971–95. Eighty-six percent of these cases were older than 50 years of age atthe time of presentation; with the majority agedbetween 60 and 70 years.

Case reports

We report seven young British patients with atopicdermatitis (AD) (six males, one female) of differentracial origins (three Asian Indian, two white Caucasian,one black West Indian and one Chinese) seen over a 4-year period with clinical features and investigative find-ings in keeping with the diagnosis of PD/AR. The meanage at diagnosis was 22 years (range 9–28 years) andthe mean age of onset of photosensitive features was18 years (range 9–23 years) (Table 1). All patients hadtotal serum IgE of >2000 units/mL, except for patient 3whose level was 310 (normal <200).

Patient 1

A 28-year-old white Caucasian female personnel officerwith AD was referred for investigation to the Photobiol-ogy Unit. At the age of 23 years she had become awareof a pruritic, swollen erythema of exposed sites, parti-cularly of the forehead and the backs of the handsoccurring after as little as half an hour of direct orwindow glass-transmitted sunlight, developing within1–2 h of exposure and followed by the appearance oftypical dermatitis. She reported that her condition wasperennial but most active in summer. At presentationshe had facial dermatitis in a photosensitive distributionaffecting the lateral sides of the forehead, the malarregions of the cheeks and the lateral aspects of the neck,with a less severe dermatitis on the trunk.

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Patient 2

A 22-year-old British Asian male student presentedwith a history of dermatitis which had first affectedhis limbs in early summer (16 months previously). Thefollowing summer he developed dermatitis of the faceand the back of the hands in addition to truncalinvolvement. When his dermatitis proved difficult tocontrol with potent topical corticosteroids, UVB photo-therapy was introduced. However, after two treatments,his skin flared. Referral for phototesting followed theobservation that his face, protected during photother-apy by a facial shield, had been spared in the generalflare. He had no history of AD but there was a strongfamily history of atopy (a sister with AD and a brotherwith asthma). Examination revealed a lichenified der-matitis which affected the back of the hands, theextensor aspect of the arms, the face with periorbitalsparing predominantly affecting the forehead, nose,malar region of the cheeks, ears and lateral neck withretroauricular sparing (Fig. 1).

Patient 3

A 21-year-old British Asian male student with AD wasreferred for phototesting. Over the previous 3 years hehad become aware of dermatitis aggravation during the

summer-time with as little as 20 min of window glass-transmitted sunlight causing a flare particularly on theforehead, sides of the neck, wrists and forearms. A trialof TL-01 UVB phototherapy resulted in exacerbationafter only three treatments and prior to phototherapyhe had a low minimal erythema dose (MED) response(<100 mJ/cm2 at 24 h). On examination, a lichenifieddermatitis of the forehead, cheeks, lateral and posteriorneck with less marked truncal involvement was evident.

Patient 4

A 21-year-old black West Indian male solderer with ADwas referred for phototesting. Over the previous 6 yearshe had become aware of sunlight-induced flares, parti-cularly on the face, sides of the neck and dorsa of thehands. He described an acute pruritic, erythematousswelling of exposed skin which could persist for up to aweek, induced by direct or window glass-transmittedlight. Five years previously, a trial of broadband UVBphototherapy was abandoned due to a marked derma-titis flare. Examination revealed a lichenified dermatitisof limbs and trunk with maximal involvement onphotoexposed areas of the face, particularly the fore-head, malar regions and lateral neck (Fig. 2) withinvolvement of the back of the hands with a markedcut-off at the wrists.

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Table 1. Patient details and positive patch test results (defined as grade 2 or greater)

Patient no. Sex/age Age at onset of atopic Past history of asthma European Standard Series, Sunscreens(years) dermatitis/onset of or hayfever/family medicaments and

photosensitivity (years) history of atopy Compositae oleoresins

1 F/28 infancy/23 yes/yes Amerchol 101 OxybenzoneRoc 16Coppertone Supershade 15Coppertone Ultrashade 25

2 M/22 21/21 no/yes ChromeCobaltNickelWool alcohol

3 M/21 infancy/18 yes/yes Chrome4 M/21 infancy/15 yes/adopted Fragrance mix Piz Buin 12

Colophony Roc 16Formaldehyde Uvistat 10

5 M/9 infancy/9 yes/yes Eusolex 8020, Eusolex 6300Sun E45 25Dundee cream L

6 M/23 infancy/22 no/yes Fragrance mix Sun E45Colophony Eusolex 6300Daisy Eusolex 8020

Piz Buin 127 M/27 9/17 no/yes Daisy

Tansy

Patient 5

A 9-year-old Caucasian boy, with asthma and severeAD, had involvement of photoexposed sites and wasreferred for investigation. A 5-year history of summerexacerbation was initially thought to be secondary toheat and sweating. The possibility of a contact orphotocontact allergy was raised after using a new sun-block. Three years previously, during a trial of TL-01UVB phototherapy, his dermatitis flared and necessi-tated hospital admission. Prior to phototesting, he hadmild dermatitis predominantly affecting the face andneck.

Patient 6

A 23-year-old British Asian male dentist had had mildAD in childhood. Eighteen months prior to presenta-tion, dermatitis had recurred on his face, neck andbacks of the hands and legs, requiring therapy withpotent topical corticosteroids. UVB phototherapy wasintroduced; however, his dermatitis deteriorated,

extending to the trunk which had previously beenunaffected. During the following summer, he noticedthat as little as 10 min of direct or window glass-transmitted sunlight could induce redness and swellingof the face and neck. He had been successfully treatedwith cyclosporin for 16 weeks. This agent was stopped2 weeks before investigation. Initial clinical assessmentrevealed a low-grade dermatitis of exposed sites of theface and ears, and patchy involvement of the limbs.

Patient 7

A 27-year-old Chinese male caterer with AD had noted10 years prior to presentation that his dermatitis dis-tribution had changed to affect sunlight-exposed sites.He felt that sunlight might have exacerbated his derma-titis but had not noticed any flare in sunshine evenwhile travelling abroad. His skin state had been difficultto control despite chronic potent topical and systemiccorticosteroid use. Examination revealed chronic liche-nified dermatitis of the backs of the hands and wrists.

Investigations

All patients were monochromator phototested, with ahigh pressure 1600 W xenon arc as the light sourceproviding a circular irradiation field of 8 mm diameterat the skin surface, to determine the action spectra,using the method previously described by Mackenzieand Frain-Bell.7 Irradiance was measured with a ther-mopile and digital voltmeter. On day 1 uninvolved skinof the back was irradiated with a series of defined dosesof the following wavebands: 305 6 5 nm, 335 6 30 nm,

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Figure 1. Lichenified papular dermatitis is seen to be greatest onphotoexposed sites.

Figure 2. Lichenified papular dermatitis on lateral neck sparing skincreases, retroauricular area and covered shoulder site.

365 6 30 nm, 400 6 30 nm, 430 6 30 nm,460 6 30 nm, 500 6 30 nm and 600 6 30 nm. Reac-tions were observed at 0, 5, 10, 15 and 30 min, and at 7and 24 h to determine minimum response doses. On day2 further radiation doses were given for each wavebandto narrow the gap between the MED and that of noresponse obtained. Results at 24 h were compared witha normal population databank.

Closed patch tests to the European Standard Seriesand Compositae oleoresins were carried out in allpatients. In addition, patch testing was conducted tothe sunscreen series and to corticosteroids in sixpatients, to the medicament series in five, to the cos-metic series in four, to tars and balsams in three, to azodyes in two and to the fragrance series in one other.Agents supplied from Trolab and Chemotechnique wereapplied on Finn Chambers, occluded for 48 h withreadings at 2 and 3 days. Results were graded 0–4,with no response graded as 0, faint but definiteerythema graded as 1, definite erythema graded as 2,erythema and oedema graded as 3 and erythema andvesicles graded as 4, with responses considered positiveif grade 2 or more. All patients required suppression ofactive dermatitis with the use of reducing potencytopical corticosteroids and nursing behind UV-protec-tive screens in our ward prior to both phototesting and

patch testing. Topical corticosteroids were discontinuedat least 5 days before testing to reduce the likelihood offalse negative results. None of the patients was onphotosensitizing drugs, antihistamines or immunosup-pressive therapy at the time of investigation.

Photopatch testing to sunscreens supplied by Chemo-technique was performed in three patients: two identicalsets were applied on Finn Chambers to the mid-upperback skin and removed after 24 h; irradiation to one setwas then performed with UVA from a metal halide source(Dr Honle lamp) with an h1 filter. The UVA dose waspredetermined by performing an MED assessment withthe same source using doses of 1, 1·5, 1·8, 2·2, 2·7, 3·3,3·9 and 4·7 J/cm2; the dosage chosen for photopatchirradiation was the one below the MED. Photopatch testreadings were performed immediately after irradiationand then at 7, 24 and 48 h postirradiation.

An erythematous phototest site was biopsied in allpatients at 48 h postirradiation. Specimens were fixed in10% buffered formalin, stained with haematoxylin andeosin and sections examined under the light micro-scope. All patients had total IgE values measured andRAST (to common inhalant and food allergens), andserological testing for antinuclear, anti-Ro and anti-Laantibodies. A porphyrin plasma scan was conducted infour of the seven patients.

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Table 2. Results of monochromator phototesting (24 h readings)

Wavelength (nm) Lowest value Minimal erythema dose (mJ/cm2 )/no response (J/cm2 )in normal

Patient 1 Patient 2 Patient 3 Patient 4 Patient 5 Patient 6 Patient 7

305 6 5 nm 27 mJ/cm2<1·8 <1·5 3·9/1·8 2·7/1·8 8·2/4·7 1/<1 3·9/1·8

335 6 30 nm 1800 mJ/cm2 120/100 <150 <180 180/150 390/330 120/100 390/330365 6 30 nm 8200 mJ/cm2 2700/1800 2200/1800 2700/1200 1500/1200 4700/3900 1000/820 1200/820400 6 30 nm 47 J/cm2 27/22 47/39 22/10 15/12 >47 10/7 47/39430 6 30 nm >82 J/cm2 56/39 >82 82/39 >82 >82 >82 >82

Figure 3. Results of monochromatorphototesting.

Results

Monochromator phototesting revealed severe broad-band UV sensitivity to UVB and UVA wavelengths inall seven patients (most marked at 305 6 5 nm and335 6 30 nm) and also to the blue/violet region (400and 430 6 30 nm) of the visible spectrum in four. Theresults of the action spectra for delayed erythema atthese wavebands (MED values were maximal at 24 h)are shown in Table 2 and Fig. 3. The morphology andhistology of the phototest site was a dermatitis in six ofthe seven patients. The skin biopsy in patient 5 wastaken from a high-dose TL-01 UVB test site (390 mJ/cm2), 10 times this patient’s MED value for TL-01(erythema present at 25 mJ/cm2—the lowest dosetested). It showed histopathological features of sunburn,which may have masked a dermatitis response.

The results of patch testing are shown in Table 1. Allpatients showed allergic responses to one or more of theEuropean Standard Series, the Compositae oleoresins,the sunscreen series and/or medicament series. Photo-patch testing to the sunscreen series in patients 1, 3 and6 failed to show evidence of photoallergy. Total IgE wasraised in all cases (>2000 units/mL in six of the sevenpatients and 310 units/mL in patient 3) and all patientshad antibodies detected to several common inhalantallergens and foodstuffs, consistent with the diagnosis ofatopy. Antinuclear, anti-Ro and anti-La antibodies werenegative in all seven patients and porphyrin scans werenegative. No patient was identified as being at high riskfor HIV infection. Testing, performed at one patient’srequest, was negative.

Discussion

It is well recognized that AD may predominantly affectthe exposed skin sites such as the hands, face and the ‘v’of the neck. It has been estimated that sunlight exposurecauses a deterioration in up to 10% of AD sufferers.8,9

Such exacerbations may be provoked by a variety offactors including heat-induced sweating8 (perhapsthrough a sweat allergy mechanism),10 concomitantidiopathic photodermatoses, drug-induced photosensi-tivity and/or sunburn (with the Koebner phenom-enon).11 Many such cases have been labelled as‘photoaggravated AD’. AD is common, and thus apercentage of PD/AR patients can be expected coinci-dentally to have AD (10% of 505 elderly PD/AR patientsinvestigated in Dundee, 1971–96).

In a previous study, monochromator phototesting in27 AD patients thought on clinical grounds to be

photosensitive, revealed normal phototest responses in13 who were considered to have heat and/or sweataggravation. Of the other 14, seven had actinic prurigo,two PD/AR, one polymorphic light eruption, three wereprobably drug-induced and the remaining patient hadunexplained borderline sensitivity to UVB.11

Over the period 1992–96, we have become aware ofa new group of young AD patients with severe, broad-band (UVB, UVA and visible wavelength) photosensitiv-ity defined by monochromator phototesting—findingsnot seen in photoaggravated AD where phototesting isnormal. These patients had clinical and morphologicaldermatitis at phototest sites combined with contactallergic dermatitis on patch testing—the diagnosticfeatures of PD/AR (CAD). A similar case of PD/AR(CAD) has been reported from Japan in a 28-year-oldman with AD. He, too, had marked photosensitivity toUVB, UVA and visible light combined with multiplecontact allergy.12 Although our study group wassmall, the proportion of higher skin type patients wassurprisingly high, an observation for which we have noexplanation.

We suggest that clinical management of this group isthe same as for older PD/AR patients.13 Definition ofresponsible wavelengths for each individual allowsappropriate UV and visible wavelength avoidanceadvice. This should include an explanation of therelevance of the effect of time of day and year, presenceof cloud, altitude and reflection from water, sand andsnow on the intensity of sunlight. Information on thepenetration of wavelengths through clothing andwindow glass helps patients to choose suitable protec-tive garments (dark coloured and thick close weave,long sleeves, and a wide-brimmed hat and gloves). Clearsticky backed plastic film which prevents transmissionof nearly all the UV wavelengths (Museum 200 Film)can be applied to window glass in cars, the home andworkplace and for severely affected patients use of alightweight UVA and UVB protective face shield maybe helpful.14 Additional advice needs to be given onavoidance of artificial sources of UV irradiation.

Patients benefit from a demonstration of the correctmode of application of sunscreens which need to bebroad spectrum, fragrance-free and of a high protectionfactor. Those with contact allergy require explanationon allergen sources to enable avoidance in the work-place and home. A regimen of reducing potency topicalsteroids will be necessary to manage acute dermatitisflares. While many find these measures sufficient, indi-vidual treatment requirements depend on severity andlife-style. Behavioural avoidance is more difficult in a

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young group and systemic immunosuppressive therapymay be necessary earlier. Patient 2 has requiredazathioprine and short courses of prednisolone overthe summer months, and patient 3 has benefited froma short course of cyclosporin and later from cautiousPUVA combined with potent topical steroid therapy.

That the presented cases are geographically dispersedthroughout Scotland and the north of England, coupledwith our experience of phototesting large numbers ofAD patients, suggests that this phenomenon in youngAD patients is uncommon. Although this new group isdescribed as having PD/AR (CAD) on the basis of thedegree and breadth of the phototest dermatitisresponses coupled with the evidence of contact allergy,it is possible in view of the young age and the as yetabsence of actinic reticuloid features, that it is a differentyet closely related condition. While spontaneous resolu-tion is a feature in some PD/AR patients,15 we have noevidence of this in the presented cases, although in timethis may emerge. Our findings underline the importanceof the phototest procedure to give a clear distinctionbetween photoaggravation and true photosensitivity.8

Acknowledgments

We thank the following consultant dermatologists whoreferred their patients for investigation: Dr R.S.Lever, DrJ.G.Lowe, Prof. R.M.MacKie, Dr C.S.Munro, DrH.M.McGrath and Dr M.J.Tidman. We also thank SisterRhoda Hodgson and Dee Watson, Senior PhotobiologyTechnician and their teams at the Photobiology Unit,Dundee for their assistance and technical expertise.

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