Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2013 May 22:2013:bcr2013009496.
doi: 10.1136/bcr-2013-009496.

Porphyria: varied ocular manifestations and management

Alok Sati  1 , Virender S Sangwan, Sayan Basu
Affiliations
Case Reports

Porphyria: varied ocular manifestations and management

Alok Sati et al. BMJ Case Rep. .

Abstract

On review of past 10 years medical records, we could find four typical cases of porphyria with rare ocular manifestations. Cases 1, 2 and 4 have presented with features suggestive of acute scleritis. Based on clinical, biochemical and dermatological evaluation, all these three cases were diagnosed to have congenital erythropoietic porphyria. Case 1 was initially managed with scleral patch graft which on subsequent melt was managed with double layered amniotic membrane grafting along with conjunctival advancement and lateral paramedian tarsorrhaphy in both the eyes. Cases 2 and 4 were managed conservatively with artificial tear drops and general protective measures. Case 3 was presented with multiple failed grafts due to repeated ulceration and infection. Owing to multiple failed grafts, Boston keratoprosthesis was done and the patient is doing well with stable kertaoprosthesis at the last follow-up visit.

PubMed Disclaimer

Figures

Figure 1
Figure 1
Right eye (A) shows an area of scleral necrosis and thinning whereas left eye (B) shows an area of scleral necrosis and uveal prolapse.
Figure 2
Figure 2
Face shows hyperpigmentation, scarring and thickening of skin.
Figure 3
Figure 3
Right eye shows an area of scleral necrosis and thinning.
Figure 4
Figure 4
Stable keratoprosthesis at the last follow-up visit.
Figure 5
Figure 5
Stable ocular surface at the last follow-up visit.
See this image and copyright information in PMC

References

    1. Venkatesh P, Garg SP, Kumaran E, et al. Congenital porphyria with necrotizing Scleritis in a 9-year-old child. Clin Exp Ophthalmol 2000;2013:314–18 - PubMed
    1. Sober AJ, Grove AS, Muhlbauer JE. Cicatricial ectropion and lacrimal obstruction associated with the sclerodermoid variant of porphyria cutanea tarda. Am J Ophthalmol 1981;2013:396–400 - PubMed
    1. Hammer H, Korom I. Photodamage of the conjunctiva in patients with porphyria cutanea tarda. Br J Ophthalmol 1992;2013:592–3 - PMC - PubMed
    1. Sati A, Sangwan VS, Basu S, et al. Boston keratoprosthesis for visual rehabilitation in porphyria cutanea tarda. BMJ Case Rep 2013 Feb 1; 2013 - PMC - PubMed
    1. Veenashree MP, Sangwan VS, Vemuganti GK, et al. Acute scleritis as a manifestation of congenital erythropoietic porphyria. Cornea 2012;2013:530–1 - PubMed

Publication types

MeSH terms