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Bullous pemphigoid — ABIM Board MCQ

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HardDermatologyBullous pemphigoidABIM Board

A 52-year-old man has fragile dorsal-hand skin, vesicles, hypertrichosis and dark urine. Urine porphyrins confirm porphyria cutanea tarda. Hemoglobin is 15.2 g/dL and ferritin 640 ng/mL; HIV and hepatitis C testing are pending. Which disease-directed treatment is preferred if no contraindication emerges?

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Correct answer: A — Begin serial phlebotomy while addressing iron and susceptibility factors

The best answer is “Begin serial phlebotomy while addressing iron and susceptibility factors”. Porphyria cutanea tarda results from hepatic uroporphyrinogen-decarboxylase inhibition and is often promoted by iron excess, alcohol, estrogen, hepatitis-C infection or HIV. In a nonanemic patient, serial phlebotomy lowers hepatic iron and porphyrin production while reversible contributors are treated. Low-dose hydroxychloroquine is an alternative when phlebotomy is unsuitable; intravenous hemin is used for acute hepatic porphyria rather than this chronic cutaneous disorder.

Reference: AASLD Clinical Liver Disease Review: Porphyria Cutanea Tarda: https://pmc.ncbi.nlm.nih.gov/articles/PMC11665602/