Ocular Lesions in Patients With Porphyria

Barnes, H. D.; Boshoff, P. H.

doi:10.1001/archopht.1952.00920010578003

Cited by 27 publications

(3 citation statements)

References 5 publications

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“…The cornea then demonstrates the formation of ulcers, perforation and scarring. These eye lesions were therefore considered to have been induced by photosensitivity 4–6 …”

Section: Discussionmentioning

confidence: 99%

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Erythropoietic protoporphyria with eye complications

Tsuboi

Yonemoto

Katsuoka

2007

The Journal of Dermatology

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We herein report a case of erythropoietic protoporphyria (EPP) complicated by a decrease in eyesight that occurred in a Japanese male. An ophthalmologist initially thought that the eyesight loss might be the result of idiopathic optic nerve atrophy due to a vascular obstruction in the fundus. There are no previous reports of EPP cases with eye complications. However, an eye abnormality has been reported in an animal model of protoporphyria after long-term, low-level exposure to blue light. As a result, in our case, it is therefore possible that a relationship may have existed between EPP and the onset of eye complications.

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“…The cornea then demonstrates the formation of ulcers, perforation and scarring. These eye lesions were therefore considered to have been induced by photosensitivity 4–6 …”

Section: Discussionmentioning

confidence: 99%

“…The relationship between EPP and the optic fundus lesion was not clear. However, some porphyria patients, such as those with porphyria cutanea tarda (PCT) and congenital porphyria (CP) do develop eye lesions 4–6 …”

Section: Introductionmentioning

confidence: 99%

Erythropoietic protoporphyria with eye complications

Tsuboi

Yonemoto

Katsuoka

2007

The Journal of Dermatology

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“…Increased levels of some porphyrins can induce phototoxic side effects in the skin (Meyer-Betz, 1913;Zalar et a / . , 1977;Grossman et a/., 1979) and the cornea, sclera and eyelid (Barnes and Boshoff, 1952;Sevel and Burger, 1971;Chumbley, 1977;Waardenburg et a/., 1961;Stokes, 1940) and retina (Freeman and Troll, 1967;Ohnishi et a/., 1983;Gomer et al, 1985;Franken et al, 1985) of the eye.…”

Section: Introductionmentioning

confidence: 99%

In Vitro Studies on the Photosensitized Oxidation of Lens Proteins by Porphyrins

Roberts

Dillon

1987

Photochem & Photobiology

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Porphyrins, which may be introduced into the eye as a result of abnormal porphyrin metabolism (uroporphyrin–Uro) or when used in the diagnosis or photodynamic therapy of certain tumors, including intraocular tumors (hematoporphyrin–Hp and‘hematoporphyrin derivative’–Hpd and mesotetra(P‐sulfonatophenyl)porphyrin–TPPS) are efficient photosensitizers in biological systems. We have been studying the potential phototoxic side effects of these drugs in the lens of the eye. Encapsulated in the human lens is a mixture of soluble protein crystallins. With little turnover of protein in the lens, any photosensitized modifications will accumulate and may result in an opacification of the lens. To evaluate the potential of different porphyrins to induce such damage, a series of porphyrins were photolyzed (transmission above 295 nm) in the presence of calf lens protein (2 mg m−1). Marked photopolymerization and histidine destruction were observed for the lens protein photolyzed in the presence of all of the drugs. We have found that the relative effectiveness of the following porphyrins to induce that damage is: Uro = TPPS Hpd = Hp. Both the singlet oxygen quencher, azide, and the free radical scavenger, penicillamine, decrease this photosensitized oxidative damage to lens protein. TPPS binds significantly to lens protein and this binding leads to conformational changes in that protein.

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Observations on Porphyria Cutanea Tarda

Brunsting¹

1954

Arch Dermatol

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Ocular Lesions in Patients With Porphyria

Cited by 27 publications

References 5 publications

Erythropoietic protoporphyria with eye complications

Erythropoietic protoporphyria with eye complications

In Vitro Studies on the Photosensitized Oxidation of Lens Proteins by Porphyrins

Observations on Porphyria Cutanea Tarda

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