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PHOTODERMATOSES - update

PHOTODERMATOSES - update. VINCENT DELEO, MD ST.LUKE’S/ROOSEVELT AND BETH ISRAEL MEDICAL CENTERS NEW YORK CITY. CONFLICTS OF INTEREST. CONSULTANT: L’OREAL ESTEE LAUDER PFIZER LIMITED BRANDS MARY KAY JOHNSON & JOHNSON GALDERMA. PHOTODERMATOSES.

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PHOTODERMATOSES - update

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  1. PHOTODERMATOSES - update VINCENT DELEO, MD ST.LUKE’S/ROOSEVELT AND BETH ISRAEL MEDICAL CENTERS NEW YORK CITY

  2. CONFLICTS OF INTEREST • CONSULTANT: • L’OREAL • ESTEE LAUDER • PFIZER • LIMITED BRANDS • MARY KAY • JOHNSON & JOHNSON • GALDERMA

  3. PHOTODERMATOSES • ABNORMAL RESPONSES TO NON-IONIZING RADIATION • UVA, uvb, visible • MECHANISM • TOXIC OR IRRITANT • CHROMAPHORE ABSORBS RADIATION AND DIRECTLY INDUCES PATHOLOGY • ALLERGIC • CHROMAPHORE ABSORBS RADIATION AND IS CONVERTED TO AN ALLERGEN • T-CELL DELAYED • IgE IMMEDIATE • INCIDENCE • UNKNOWN OVERALL- PMLE 10% IN USA

  4. PHOTODERMATOSES - CLASSIFICIATION • IDIOPATHIC • POLYMORPHOUS LIGHT ERUPTION • CHRONIC ACTINIC DERMAITIS • SOLAR URTICARIA • HYDROA VACCINIFORME AND ACTINIC PRURIGO • GENETIC AND METABOLIC • PORPHYRIAS • PEDIATRIC PHOTOSENSITIVITY SYNDROMES • EXOGENOUS CHEMICAL • PHOTODRUG REACTIONS • PHOTOIRRITANT CONTACT • PHOTOALLERGIC CONTACT • PHOTOAGGRIVATED • LE & DM • PEMPHIGUS & PEMPHIGOID • DARIER’S

  5. Photodermatoses- PatternsWhat you need to know • Major Patterns – Common Conditions • Acute and Recurrent • Immediate – Short Lived – Minutes to Hours • Delayed – Persistent - Days • Chronic • Eczematous • Bullous • Pigmented • Plaques - Psoriasiform

  6. PATIENT 1 • 35 y/o Caucasian Female • Three month history of an eruption that occurs while she is in the sun and persists for about one hour. Itchy. Blotchy red. • Involves all sun exposed areas. Sometimes spares the face. • Variable amount of exposure necessary to cause the eruption.

  7. PATIENT 1 Recurrent -Immediate – Short Lived • Differential diagnosis • SOLAR URTICARIA • Usually UVA and/or Visible • R/O Lupus • Rare – ANA, anti-Ro, La • Psychosomatic disease? • If they tell you the light from the lamp in your office can cause the eruption but the sun doesn’t! • Work-up • Photo testing with immediate reading – not always positive

  8. SOLAR URTICARIA • IDIOPATHIC • INCIDENCE • 3.1 per 100,000 in SCOTLAND beattie 03 • GENDER • 60-70% female SCOTLAND & SPAIN lasa 05 : 21% in SINGAPORE chong 04 • AGE AT ONSET • YOUNG ADULTS

  9. ETIOLOGY • IMMEDIATE ALLERGIC RESPONSE • IgE MEDIATED • ANTIGEN – PRODUCED BY UV • CIRCULATING – PASSIVE TRANSFER POSSIBLE • TYPE I • GENERATED ONLY IN PATIENTS • TYPE II • GENERATED IN PATIENTS AND CONTROLS • CUTANEOUS IN ORIGIN

  10. TREATMENT • PHOTOPROTECTION • SPECTRALLY DETERMINED • H1 ANTIHISTAMINES • HIGHER DOSES • COMBINATIONS • FEXOFENADINE & DOXEPIN • PHOTOTHERAPY • DESENSITIZATION WITH SUB-MUD DOSES • CAREFULLY – NOT LONG LASTING • PUVA • NATURAL SUN EXPOSURE • PLASMAPHERESIS • ONLY IF CIRCIRULATING FACTOR IS PRESENT • CYCLOSPORINE • IV Ig • Omalizumab

  11. PATIENT 2 • 28 y/o Caucasian female • Two years ago was in Aruba !! And after the third day developed an itchy rash that involved the arms and the chest and lasted for a week. Spared the face. • Raised bumps. Went to ER – Ruined her vacation. Happened again in Las Vegas. Does not happen at home. • Getting married and wants to go back to Aruba for vacation. • She thinks it was the sunscreen.

  12. PATIENT 2Recurrent - Delayed - Persistent • Differential diagnosis • PMLE • Spares the face • Photoallergic Contact Dermatitis • Involves the face • Lupus – Rare – ANA, anti-Ro,La • Work Up • MEDs and Photopatch Tests – negative • Skin Biopsy

  13. What you need to know • PMLE is the most common photodermatosis that you will see in your office. And you probably won’t ever see the rash. It is a diagnosis of exclusion.

  14. PMLE - EPIDEMIOLOGY • MOST COMMON PHOTOSENSITIVITY • FEMALES>MALES at least 2:1 • ONSET IN YOUNG ADULTS - 20’s in women and somewhat older in men • INCIDENCE • USA - 10% • SWEDEN - 21% • UK - 15% • AUSTRALIA - 5%

  15. PMLE - MORPHOLOGY • SMALL PAPULAR OR PAPULO- VIESICULAR • LARGE PAPULE OR PLAQUE • Facial involvement • May be TUMID LE • vesiculo-bulbous • hemorrhagic • erythema multiforme-like • IN SUN-EXPOSED SKIN

  16. PHOTOBIOLOGICAL STUDIES • MED’S - WNL • PHOTOPATCH TESTS -WNL • PHOTOPROVOCATION TESTING • HIGH INTENSITY - MULTIPLE INSULT TESTING • UVB • UVA • BOTH • UVA+/-UVB MOST COMMON

  17. PMLE - HISTOLOGY • DENSELY AGGREGATED PERIVASCULAR DERMAL LYMPHOCYTIC INFILTRATE • VARYING DEGREES OF SPONGIOSIS • NORMAL DIRECT IMMUNOFLUORESCENSE

  18. HISTOLOGICAL DIFFERENTIAL DIAGNOSIS • LE • LYMPHOCYTIC INFILTRATE OF JESSNER • CUTANEOUS LYMPHOID HYPERPLASIA • PMLE

  19. PMLE - ETIOLOGY • DELAYED HYPERSENSITIVITY? • CD4+ Tcells EARLY - CD8+ LATE • INCREASED LANGERHANS CELLS • RESISTANCE TO UVB wackernagel 04 • INCREASED ICAM-1 – KERATINOCYTES • UVB DECREASED NEUTROPHIL-INDUCED IMMUNOSUPPRESSION schornagel 04 • UVB DECREAESED TNF-alpha, IL-4, IL-10 • TOXIC RESPONSE – NORMAL VARIANT? • LARGE AREAS NECESSARY FOR REPRODUCTION • L0W RECURRENCE RATE

  20. PMLE -ETIOLOGY • GENETIC • HLA TYPING NORMAL 41 pts - lane 92 • FAMILIAL CLUSTERING – millard 2000 • 21% mono twins – 18% dizy twins • A FORM OF LUPUS ERYTHEMATOSUS? • 142 pts ++ANA/ RO/LA- 10% : fulfill criteria for LE - 3% murphy 92 • 138 pts followed for 32 yrs - 15% developed some immune disease but only 2% developed LE - hasan 98 • PMLE by history in 49% of 337 LE pts - nyberg 97

  21. PMLE - TREATMENT • SUNSCREENS - PHYSICAL BLOCKERS, AVOBENZONE AND ECAMSULE • Cutis. 2009 • TOPICAL ALPHA-GLYCOSYLRUTIN • BETA-CAROTENE • ANTI-MALARIALS • NICOTINAMIDE • OMEGA-3 FATTY ACID DIET • PHOTOTHERAPY - NBUVB

  22. PROGNOSIS • FEW LARGE STUDIES • SCOTLAND – 87 pts – F/U <1-24YRS • RESOLUTION • 5 YEARS – 12% • 10 YEARS – 26%

  23. PATIENT 3 • 28 y/o Caucasian female • Two years ago was in Aruba !! And after the third day developed an itchy rash that involved the arms and the chest and lasted for a week. INVOLVED THE FACE. • Raised bumps. Went to ER – Ruined her vacation. MAY HAPPEN AT HOME. • Getting married and wants to go back to Aruba for vacation. • She thinks it was the sunscreen.

  24. RECURRENT – DELAYED - PERSISTENT

  25. PATIENT 3Recurrent - Delayed - Persistent • Differential diagnosis • PMLE • Spares the face • PHTOALLERGIC CONTACT DERMATITIS • Involves the face • Lupus – Rare – ANA, anti-Ro,La • Work Up • MEDs (WNL) and Photopatch Tests (+)

  26. PHOTOPATCH TEST PROTOCOL NACDG* • DAY 1 • MED UVA, UVB, VIS (READ for URTICARIA) • APPLY TWO SETS OF ANTIGENS • DAY 2 • READ MEDS • DAY 2 OR 3 • REMOVE PATCHES AND READ • IRRADIATE - 10J/cm2 - ONE SET • DAY 4 OR 5 • READ • DAY 6 through 10 • READ • *VERY SIMILAR TO LIM 2003 AND EUROPEANTASK FORCE FOR PHOTOPATCH TESTING 2004

  27. NACDG

  28. What you need to know • If its photoallergic contact dermatitis it will be due to sunscreens almost always. If you can photopatch test to confirm the diagnosis (and you should) but if you can’t then use physical blockers (titanium and zinc) only. Remember however that sunscreens are used in many non-sunscreen agents like shampoos and remember that there can be short term persistent light reactivity.

  29. PATIENT 4 • 56 Y/O Caucasian woman with a three month history of an itchy rash on dorsal hands and face. Rash responds slightly to topical steroids but is chronic. • Patient does not relate the eruption to specific episodes of exposure. • Patient is almost clear in the winter in New York. • Patient has diabetes and hypertension and is on Insulin and hydrochlorothiazide.

  30. CHRONIC E RUPTIONS – IN SUN EXPOSED DISTRIBUTION • Photodrug Reaction • Photodistributed and history of drug • Chronic Actinic Dermatitis • Rarer with predisposing factors • Photoallergic Contact Dermatitis • Very rare to be chronic • Contact Dermatitis (Airborne) • Usually plants in outdoor workers

  31. PHOTODRUG REACTIONS • MOST ARE UVA - SOME UVB • MOST ARE TOXIC - FEW ALLERGIC? • MORPHOLOGY IS VARIABLE • MED’S MAYBE ABNORMAL • PHOTOPATCH MAY BE POSITIVE • CLINICAL DIAGNOSIS

  32. PATIENT 5 – CHRONIC - OTHER MORPHOLOGY • Any age, any race • Rash on exposed areas • History of exposure to photosensitizing drug • Morphology diverse • Eczematous • Bullous • Erythematous • Hyperpigmented • Psoriasiform • Lichenoid

  33. PHOTODRUG REACTION THIAZIDE

  34. PYROXICAM erythematous

  35. CHLOROPROMAZINE lichenoid

  36. LICHENOID THIAZIDE

  37. AMIODARONE hyperpigmented

  38. Clinical Photos • DILTIAZEM hyperpigmentation

  39. DRUG INDUCED SCLE THIAZIDE

  40. PSEUDOPORPHYRIA NSAID’s

  41. SKIN CANCER VORICONAZOLE photosensitivity with Melanoma and NMSC

  42. PATIENT 6 – CHRONIC ACTINIC DERMATITIS • 50 y/o African American man with a two year history of a rash on dorsal hands and face. Itchy and partially responsive to topical steroids. Better in the winter. • HIV positive for 10 years • Multiple drugs

  43. HIV

  44. CHRONIC ACTINIC DERMATITIS • INCIDENCE 1:6000 (Scotland) • MALE:FEMALE 5-10:1 • MEAN AGE - 65Y/O • RACE - WHITE but also in BLACKS, ASIANS • OUTDOOR WORKERS

  45. PREDISPOSING FACTORS • PHOTOALLERGIC CONTACT DERMATITIS • PHOTODRUG REACTIONS • CONTACT ALLERGY TO WORKPLACE CHEMICALS - CHROMATES • AIRBORNE CONTACT DERMATITIS • ATOPIC DERMATITIS – YOUNG ADULTS • HIV

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