63530 Pseudoporphyrias: A Systematic Review and Meta-analysis

Document Type

Conference Proceeding

Publication Date

9-1-2025

Publication Title

J Am Acad Dermatol

Abstract

Pseudoporphyria, a bullous disorder visually and histologically identical to porphyria cutanea tarda, is distinguished by normal porphyrin levels on biochemical testing. Bullae, vesicles, skin fragility, and scarring arise on the skin in a photodistributed pattern from various triggers. We present a systematic review of pseudoporphyria cases across different patient demographics. Of the 477 articles describing pseudoporphyria identified in three databases, 138 articles describing 191 patients were analyzed. Cases of pseudoporphyria were induced by drugs (136), renal insufficiency (38), UV-exposure (14), and miscellaneous triggers (lime juice, brewer’s yeast, and Coca-Cola) (3); most cases resolved with trigger discontinuation. Of the few treated (5.1%), photoprotective agents (4.7%) and topical steroids (3.7%) were most common. Hands were the most affected area (78.5%). In drug-induced cases, NSAIDs were most commonly implicated (57.4%), followed by antifungals (7.4%). Therapy was discontinued in the majority of drug- induced cases (90.4%); complete symptom resolution occurred in 80.1%. For renal insufficiency- induced cases, 97.4% were on dialysis, predominantly hemodialysis. Therapy was discontinued in only 7.9%, however full resolution occurred in 73.7% of patients. Interestingly, 100% of UV radiation-induced cases were biopsy-proven. While rare, pseudoporphyria is an important consideration when patients present with a vesiculobullous rash. Thorough review of comorbidities, medications, and recent exposures is essential for trigger identification. A genetic predisposition for pseudoporphyria may be considered, highlighted by a case of monozygotic twins exhibiting identical symptoms after UV-exposure. Further research regarding genetic predisposition and family history, mechanisms behind triggering agents, and diagnostic standards may aid in developing targeted preventive strategies and treatments.

Volume

93

First Page

AB276

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