Porphyria Cutanea Tarda (P.C.T.)

on 27.2.08 with 0 comments



Description and Symptoms

Photodistributed 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.

Pathology

May 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 Therapy

Complete sun protection extremely important. Treatment of underlying disorder. Phlebotomy and low-dose antimalarials (hydroxychloroquine) are treatment options.


Category: Dermatology Notes

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