SEMINAR

APPROACH TO A PATIENT WITH     PHOTOSENSITIVITYPHOTOSENSITIVITY

PRESENTER DR PANKAJ CHATURVEDIPRESENTER  ‐ DR PANKAJ CHATURVEDIMODERATOR‐ DR SOMESH GUPTA

ALL INDIA INSTITUTE OF MEDICAL SCIENCES (AIIMS)ALL INDIA INSTITUTE OF MEDICAL SCIENCES (AIIMS)

Feedback will be appreciated drpankaj4u@gmail.com

PhotosensitivityPhotosensitivity

Abnormal cutaneous  response to ordinarylight exposure .

Causes ) h b d d1)Idiopathic photodermatoses

Polymorphous light eruption (PMLE)

4)Photoexacerbated dermatosesAutoimmune diseases

Lupus erythematosusActinic prurigoHydroa vacciniformeChronic actinic dermatitis

DermatomyositisPemphigusBullous pemphigoid

Solar urticaria

2)Secondary to exogenous agents

Pemphigus erythematosusGenodermatoses

Hailey‐Hailey diseasePhotoallergyPhototoxicity

3)Secondary to endogenous 

Darier,s diseaseBloom syndromeRothmund‐Thompson syndrome) y g

agentsPorphyrias

p yKindler syndromeCockayne,s syndromeXeroderma pigmentosump gTrichothiodystrophyHartnup disese

CausesInfections

Herpes simplexViral exanthems

Other dermatological disordersAtopic dermatitisAcneViral exanthems

Verruca planaNutritional deficiencies

Pellagra

Grover,s diseaseDisseminated superficial actinic porokeratoses

PellagraPyridoxine deficiency

Lichen planusPsoriasisReticular erythematousm cinosis (REM) s ndromemucinosis (REM) syndromeRosaceaCutaneous T‐cell lymphomaE th ltifErythema multiformeGranuloma annulareJessner,s lymphocytic infiltratePi i i b il iPityriasis rubra pilarisSeborrhoeic dermatitis

When to suspect a photosensitive disorder?photosensitive disorder?

Which sites are involved ?Which sites are involved ?

Which sites are not involved ?Which sites are not  involved ?

HistoryHistory

Age of onsetAge of onset

I f t d t ddl S h l i hildInfants and toddlers

Genodermatoses

School going children

Polymorphous light eruptionErythropoietic 

porphyrias

N t l/ Childh d

eruption

Hydroa vacciniforme

A ti i i ( i l )Neonatal/ Childhood LE

Actinic prurigo (girls)

SLE

Juvenile dermatomyositis

Age of onsetAge of onset

Ad lt Eld lAdults

Polymorphous light eruption

Elderly

eruption

Solar urticaria

D i d d

Chronic actinic dermatitis

D i d dDrug induced photosensitivity

P h i t

Drug inducedphotosensitivity 

Porphyria cutanea tarda

Lupus erythematousLupus erythematous

Symptoms

Itching Burning pain ‐ Erythropoietic porphyriaBurning pain                  Erythropoietic porphyria

Occular symptoms       ‐ Actinic prurigo

H d i if‐ Hydroa vacciniformeMucosal involvement  ‐ Actinic prurigo

Pellagra‐ Pellagra‐ SLE

Systemic symptoms ‐ Solar urticariaSystemic symptoms       Solar urticaria‐ Porphyria‐ SLE‐ Pellagra  

Relation to sun exposurep

Latent interval between exposure and eruptioneruption

Few minutesSolar urticariaDrug induced like amiodarone

Upto few hrsPLE Hydroa vacciniformeHydroa vacciniformeDrug induced (thiazides)Hydroa vacciniformisEPPSCLE

Relation to sun exposureRelation to sun exposure

T d fType and amount of sunexposure

Prolonged exposure after a long gap ‐ PMLETanning beds (UVA)

Relation to season

Early  part of the sunny season and becomes less severe as the season progresses – PMLEp g

Does rash comes in episodes?

Is patient completely asymptomatic inbetween episodes?Yes ‐ PhotodermatosesNo ‐ Photo exacerbated dermatoses

Duration  of the persistence of the lesions in the absence of additional sunexposureSubside in hours → solar urticariaSubside in days to wks → PMLEPersist wks to months/throughout the

CAD PCTseason → CAD, PCT

Patient described morphology of the lesionsPatient described morphology of the lesionsWheals

Erythema

Blisters

Papules

Scarring

Whether lesions occur by window glassWhether lesions occur by window glass filtered sunlight ?

Yes UVAYes – UVALesions occuring/worsening despite sunscreen applicationsunscreen application 

Drug historyDrug history

Hi f d hi h h kHistory of drugs which pt has taken

History of drugs which patient has appliedHistory of Desi/Homeopathic/Ayruvedic medication eg Bagchi

History of other over the counter preparations which patient may not consider medications/drugs

History of cosmetics/ perfumes

Photosensitizing Agents

Occupational historyOccupational history

Exposure to sun, artificial light sources Handling of plants drugs and chemicalsHandling of plants, drugs and chemicals

Family history

GenodermatosesPorphyria

PMLE (20%)

Actinic prurigo (20%)p g ( )

ExaminationExamination

Distribution of the lesionsI l t f S i fInvolvement of ForeheadBridge of nose

Sparing ofBelow  the eyebrowsunder the hair fringeBridge  of nose

Upper cheeksChin

under the hair fringeon the upper eyelids below the noseChin 

Helix of the earBack and sides of the

below the noseUpper lip  Behind the earlobesBack  and sides of the 

neckV area of neck

Behind  the earlobesDistal phalynx & webspaces of the 

Dorsa  of the hands and feet

fingersskin folds

Extensor extremities

Macules/PapulesMacules/Papules

PMLEPMLE

LEAP

Drug eruption

AP AP AP

PMLEPMLE

Drug induced photosensitivity

Erythematous edematous plaquesErythematous edematous plaques

PMLEPMLE

LEDM

Porphyria

PMLE PMLE PMLE

SLE Seb. Dermatitis

DM

DM

PCT

Eczematous plaquesEczematous plaques

CADCAD

AIDSThiazides

Photosensitive atopic dermatitis

CAD Photoallergic  CD

HIV Pt with drug induced photosensitivityCAD

PellagraPellagra

VesiculobullousVesiculobullous

H d i ifHydroa vacciniforme

PorphyriaJuvenile Spring Eruption

Phototoxic CD

Drugs (Frusemide, Nalidixic Acid, )

HV

Juvenile spring eruption

PCTPhototoxic der. d/t topical 

PsoralensPsoralens

Phytophotodermatitis

Lichenoid lesionsLichenoid lesions

CADCAD

Actinic ReticuloidActinic LP

Drugs (Thiazides)

ARAR

TelangiectasiasTelangiectasias

RRosacea

XPAtaxia telengiectasis

Bloom syndrome

SLEDMDM

Drugs (ACE inhibitor, Nifedipine, Amlodipine)

Rosacea

Bloom syndrome

HyperpigmentationHyperpigmentation

M lMelasma

Berloque dermatitisPellagra 

Melasma Berloque derm

ScarringScarring

P h iPorphyria

HV

HypertrichosisHypertrichosis

P h iPorphyria

Investigations

Ph iPhototesting

PhotopatchHistopathology

Other lab. Studies

PhototestingPhototesting

N i d f di i il di i iNot  required for diagnosis until diagnosis is uncertain

l h lPrimarily a research tool

PhototestingPhototesting

M h i h iMonochromatic phototesting

Photoprovocation

PhototestingMonochromatic phototestingMonochromatic phototesting

Wavelength  dependency of the disorder & to elicit the eruption when possiblethe eruption when possible Exposure (covered areas) to a series of doses of UVR to determine the MED (Xenon arc irradiationto determine the MED (Xenon arc irradiation monochromator )

C i ith th f lt f th lComparison  with the range of results for the normal population (by MED chart)

MED b l th l li it f lMED below the lower limit of normal 

↓Photosensitivity present

Monochromatic phototestingp g

PhototestingPhototestingPhotoprovocation testingT i d th l i f li i l di i /biTo induce the lesion for clinical diagnosis/biopsySolar stimulator (Xenon arc filtered)L f ki k t b t bl fLarge areas of skin known to be succeptable for eruptionIrradiation for 2 3 consecutive days may be reqdIrradiation for 2‐3 consecutive days may be reqd.Almost always +ve in solar urticaria, in minutesVariable +ve in PMLE (upto 50% +ve if consecutiveVariable +ve in PMLE (upto 50% +ve if consecutive for 2‐3 days)Cant discriminate from other photodermatosesCant discriminate from other photodermatoses

Photoprovocation testingp g

PhotopatchPhotopatch

I di tiIndicationEczematous eruption in photodistribution

Photoallergic dermatitis

CAD (Photosensitivity dermatitis/ Actinic reticuloid syndrome)reticuloid syndrome)(phototests also positive)

Photopatch series

5 Bromo 4’chlorosalicylanilide 1% Camphor 10%5‐Bromo‐4 chlorosalicylanilide 1%Hexachlorophene 1%Bithionol 1%

Camphor 10%2‐phenyl‐5‐benzimidazolsulphonic acid 10%Oxybenzone 10%Sulfanilamide 1%

Promethazine hydrochloride 1%Quinidine sulphate 1%

Oxybenzone 10%Thiourea 0.1%Olaquindox 1%

Fragrance mix 1%para‐Aminobenzoic acid 10%2‐Ethylhexyl‐p‐

Parthenium 1:100,1:200 (acetone)Xanthium (Aq)

2 Ethylhexyl pDimethylaminobenzoate 10%Benzophenone‐4 10%4‐tert‐butyl‐4’‐Methoxy‐

Chrysanthemum (Aq)Fentichlor6‐methyl coumarin4 tert butyl 4 Methoxy

DibenzoylmethaneIsoamyl p‐methoxycinnamate10%

BenzophenoneParthenium hysterophorusParaphenylenedimaine

2‐Ethylhexyl‐p‐methoxycinnamate 10%

ParaphenylenedimainePetrolatum (control

Day 1 –Perform MED testing Apply duplicate sets ofDay 1 Perform MED testing. Apply duplicate sets of photoallergens on left and right back

Day 2 – Read MEDs Irradiate one set of allergens with UVADay 2 – Read MEDs. Irradiate one set of allergens with UVA (10 mJ/cm2 or 50% of MED‐A,whichever is less), covering the other with an opaque materialmaterial

Day 3 –Remove nonirradiated patches and perform first reading of reactions to both sets of photoallergensreading of reactions to both sets of photoallergens(both sites)

f f b h fDay 5 – Perform second reading of reactions to both sets of photoallergens

International Contact Dermatitis Research Group Scoring System

D b f l i (f i h l )± Doubtful reaction (faint erythema only)

+ Weak positive reaction (erythema, infiltration,   bl l )possibly papules)

++ Strong positive reaction (erythema, infiltration,   papules, vesicles)

+++ Extreme positive reaction (intense erythema, infiltration, coalescing vesicles or bulla

IR Irritant reactionNT Not tested

German/ Swiss / Austrian

0   no erythema1+ erythema2+ erythema, infiltration, +/‐papule2  erythema, infiltration,  / papule3+ erythema, papule, vesicle4+ erythema blister erosion4+ erythema,blister, erosion

ResultResult

Reading of the photopatch testReading of the photopatch test

Diagnosis                                  Irradiated site                              Unirradiated siteg

No sensitivity                                    ‐ ‐Photocontact allergy                       +                                                             ‐Contact allergy + +Contact allergy                                 +                                                             +Photocontact & contact allergy ++                                                          +

Results in CADResults in CAD

Phototests PhotopatchPhototests        Photopatch

Persistent light reactors UVB+UVA+/‐VR     PCD

Photosensitive eczema UVB                            ‐

Photosensitivity Dermatitis UVB+UVA+/‐VR     +/‐

HistopathologyHistopathology

PMLEPMLE

+/ spongiosis dyskeratosis+/‐ spongiosis, dyskeratosis, exocytosis, basal cell vacuolization

Tight perivascular infiltrate in upper dermis and middermis (T ll )cells)

Upper dermal and perivascular edema

Endothelial cell swellingEndothelial cell swelling

Actinic prurigoActinic prurigo

A th i t iAcanthosis,exocytosis spongiosis

Lymphohistiocytic dermal perivasculardermal perivascular infiltration 

Hydroa vacciniformeHydroa vacciniforme

I id l i lIntraepidermal vesicle 

Focal keratinocyte necrosis Spongiosis

Dermal perivascular neutrophilic and lymphocytic infiltration

Vasculitis+/‐

Solar urticariaSolar urticaria

Dermal vasodilationDermal vasodilation and edema

Mild  interstitial and P/V inflammatory cell infiltrate of L & Einfiltrate of L & E

Chronic actinic dermatitisChronic actinic dermatitis

E id l i iEpidermal spongiosis, acanthosis

Perivascular lymphocytic cellularlymphocytic cellular infiltrate , confined to the upper dermis pp

Actinic reticuloidActinic reticuloid

Marked acanthosisMarked acanthosis

Mimic cutaneous T‐cell l hlymphomaPautrier‐like microabscesses(rare) Dense epidermotropicinfiltrateSometimes  hyperchromaticconvoluted nuclei and giantconvoluted nuclei and giant cellsNo marked increase in mitosesmitoses

Phototoxic reactionsPhototoxic reactions

N i f k ti tNecrosis of keratinocytes

I t id l bli tIntraepidermal blister

Epidermal necrosisEpidermal necrosis

SpongiosisSpongiosis

Sparse dermal infiltrateSparse dermal infiltrate.

Porphyria cutanea tardaPorphyria cutanea tarda

S b id l bli tSubepidermal blister with minimal or no infiltrateinfiltrate

FestooningFestooning

Other lab. TestsOther lab. TestsANA                  If clinical suspicion of LE

Anti Ro/SsaAnti La/SSb

Urine, stool, blood porphyrin estimation

Blood film fluorescence 

RBC protoporphyrin

Control Positive

Autologus serum test in SUAutologus serum test in SU

HLA‐typing(HLA‐DR4, HLA‐DRB1*407 in actinicHLA typing(HLA DR4, HLA DRB1 407 in actinic prurigo)DNA repair studies in fibroblast culture

Drug and chemical phototoxicity studies

TreatmentTreatment

Ph i Cl hi HPhotoprotection – Clothing, Hats

ANDSunscreens

Symptomatic treatment

PMLE

Actinic PrurigoActinic Prurigo

Hydroa vacciniformeHydroa vacciniforme

Solar urticariaSolar urticaria

CADCAD