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Description and SymptomsPhotodistributed blisters, crusts and erosions that lead to scars and pigmentary changes. May be associated with milia, hypertrichosis and sclerodermoid changes.
Diagnosis Clinical suspicion confirmed by urine or serum porphyrins.
PathologyMay be associated with viral hepatitis, hormonal therapies, hemodialysis or alcohol intake. Etiology is a defect in the uroporphyrinogen decarboxylase enzyme used in the production of heme.
Principles of TherapyComplete sun protection extremely important. Treatment of underlying disorder. Phlebotomy and low-dose antimalarials (
hydroxychloroquine) are treatment options.
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Dermatology Notes
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