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Photosensitive disorders: Clinical features

Photosensitive disorders: Clinical features
  Sex Age of onset Timeline of eruption Location Common lesion morphology and symptoms Family history Action spectrum Phototesting Laboratory findings Duration/
resolution
Key features
Polymorphous light eruption F>M First 3 decades Hours after sun exposure, lasts days to weeks; most conspicuous in spring; improves during summer Sun-exposed areas Pruritic papules, papulovesicles, plaques. May be present UVA, UVB, visible Normal MED; provocative phototesting may induce lesions.   Recurrent over the course of years, may improve over time Most common photodermatosis; "hardening" phenomenon may occur.
Juvenile spring eruption M>F Primarily childhood; also may be seen in young adults As with polymorphous light eruption Classically, helices of ears Erythematous papules, bullae. May be present UVA? As with polymorphous light eruption.   Recurrent over years, may improve with age  
Actinic prurigo F>M, M>F reported in adult onset in Asia Childhood; adult onset may occur in Asians Persistent during summer; may also be present year round Sun-exposed areas, may also affect unexposed areas Pruritic papulonodules, crusts, excoriations, lichenification, cheilitis. Yes, in up to 50% UVA>UVB 60% with reduced MED, 60 to 70% with positive provocative phototests.   Improves in adolescence, but also may persist More common in American Indians and Mestizos. May have ocular findings.
Hydroa vacciniforme Slight M>F Childhood Hours after sun exposure Face, dorsal hands Erythematous macules, papules, vesicles, crusts. Rarely positive Likely UVA May show reduced MED to UVA in some cases.   Usually resolves by adolescence/young adulthood, but may persist Lymphoproliferative disease association with severe cases.
Chronic actinic dermatitis M>F Older, but may be seen in younger patients Persistent; worsens in summer, may have findings year round Sun-exposed areas Eczematous patches, lichenification, may see palmoplantar involvement.   UVA, UVB, visible light Decreased MEDs to UVA, UVB, or visible light. May see Sézary cells in severe cases Persists for years, may resolve Often coexistent contact dermatitis.
Solar urticaria F>M Young or mid-adulthood Appears within minutes, individual lesions resolve within 24 hours Sun-exposed areas Urticarial plaques (hives), occasional systemic symptoms.   UVA, UVB, visible light Evaluated with MUD.   Persists for years, may resolve "Hardening" phenomenon may occur.
Phototoxicity M = F Any age Appears within hours of sun exposure, can occur after first dose of drug Sun-exposed areas Exacerbated sunburn appearance.   UVA In systemic phototoxicity, MED for UVA decreased, UVB and visible light MEDs normal.   Resolves when drug discontinued and cleared from body Can occur in anyone.
Photoallergy M = F Any age Appears 1 to 2 days after exposure to sun and inciting agent in sensitized individual Sun-exposed areas Pruritic, eczematous lesions.   UVA

In systemic photoallergy, MED for UVA decreased, UVB and visible light MEDs normal.

Occasional drugs have reduced MED to UVB rather than UVA.
  Resolves with discontinuation of inciting agent Usually due to topical agents.
Erythropoietic protoporphyria M = F Onset in early childhood; rarely in adults with clonal bone marrow disorders Symptoms may begin within minutes of sun exposure; worse in spring and summer, improves in winter Nose, cheeks, hands Acute prodromal burning pain, itching, stinging; continued exposure followed by severe pain, swelling, systemic symptoms. Skin findings absent or mild scarring, leathery changes on knuckles or face. Variable, autosomal recessive, often skips generations Soret band (400 to 410 nm) Often normal, some patients may note stinging sensation or lesions. Markedly elevated erythrocyte protoporphyrin (approximately 85 to 100% metal-free) Lifelong Altered behavior and impaired quality of life. May develop liver disease, which may be severe.
X-linked protoporphyria M>F Onset in early childhood; rarely in adults with clonal marrow disorders Symptoms may begin within minutes of sun exposure; worse in spring and summer, improves in winter Nose, cheeks, hands Acute prodromal burning pain, itching, stinging; continued exposure followed by severe pain, swelling, systemic symptoms. Skin findings absent or mild scarring, leathery changes on knuckles or face. Variable, X-linked inheritance Soret band (400 to 410 nm) Often normal, some patients may note stinging sensation or lesions. Markedly elevated erythrocyte protoporphyrin (approximately 50 to 85% metal-free) Lifelong Altered behavior and impaired quality of life. May develop liver disease, which may be severe.
Porphyria cutanea tarda M>F Middle age, may occur earlier Lesions develop subsequent to and may seem unrelated to light exposure Dorsal hands, face, feet and other sun-exposed areas Chronic, mostly painless skin fragility, bullae, crusts, scarring, hypertrichosis, hyper- and hypopigmentation, sclerodermoid changes. Uncommon; approximately 20% have heterozygous UROD mutations with low penetrance; some have HFE (hemochromatosis) mutations Soret band (400 to 410 nm)   Elevated urine and plasma porphyrins (mostly uroporphyrin and heptacarboxyl porphyrin) Chronic; readily treated by phlebotomy, low-dose hydroxychloroquine or antivirals for hepatitis C Iron-related disorder with combinations of contributing factors, either acquired (alcohol, smoking, hepatitis C, estrogens) or inherited (UROD and HFE mutations).
Pseudoporphyria F>M Any age; 10% of children taking naproxen   Sun-exposed areas Skin fragility, bullae, crusts, scarring, milia. Negative UVA   Normal porphyrin levels Symptoms resolve with sun protection, avoidance of tanning bed use, and discontinuation of the causative medications May be caused by medications, renal failure and hemodialysis, and excessive tanning bed use.
F: female; M: male; MED: minimal erythema dose; MUD: minimal urticarial dose; UVA: ultraviolet A; UVB: ultraviolet B.
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