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'Poe-phyria,' Madness, and The Fall of the House of Usher

Leland S. Rickman, MD; Choong R. Kim, MD
JAMA. 1989;261(6):863-864. doi:10.1001/jama.1989.03420060067030.
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To the Editor.—  Many explanations for the malady that affected Roderick and Madeline Usher in Edgar Allan Poe's The Fall of the House of Usher1 have been proposed, mostly psychiatric in nature.2 One explanation for their affliction that has not been entertained is porphyria. Poe described an "acute bodily illness" and a "mental disorder" with intermittent exacerbations consistent with porphyria in Roderick Usher, who suffered from a familial disorder that involved his appearance, behavior, and sensory and motor function.Most porphyrias are transmitted in an autosomal-dominant fashion. Roderick Usher, his twin sister Madeline, and other members of the Usher family were afflicted by a familial disorder—"a constitutional and a family evil." Hypertrichosis and hirsutism, seen in some of the porphyrias, are described in the physical appearance of Roderick Usher: "Hair of a more than web-like softness and tenuity... above the regions of the temple... the silken hair... to

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