approach to photosensitivity - dr pankaj aiims, new delhi
DESCRIPTION
How to approach a patient presenting with photosensitivity.TRANSCRIPT
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 [email protected]
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