Porphyria: varied ocular manifestations and management

Sati, Alok; Sangwan, Veena; Basu, Sayan

doi:10.1136/bcr-2013-009496

Cited by 8 publications

(5 citation statements)

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“…Additionally, conjunctivitis may be associated with systemic conditions, including immunerelated diseases [e.g., Reiter's, Stevens-Johnson syndrome (SJS), and keratoconjunctivitis sicca in rheumatoid arthritis], nutritional deprivation (vitamin A deficiency), and congenital metabolic syndromes (Richner-Hanhart syndrome and porphyria) [4,5] (Table 1).…”

Section: Introductionmentioning

confidence: 99%

Conjunctivitis: A Systematic Review

Azari

Arabi

2020

JOVR

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Conjunctivitis is a commonly encountered condition in ophthalmology clinics throughout the world. In the management of suspected cases of conjunctivitis, alarming signs for more serious intraocular conditions, such as severe pain, decreased vision, and painful pupillary reaction, must be considered. Additionally, a thorough medical and ophthalmic history should be obtained and a thorough physical examination should be done in patients with atypical findings and chronic course. Concurrent physical exam findings with relevant history may reveal the presence of a systemic condition with involvement of the conjunctiva. Viral conjunctivitis remains to be the most common overall cause of conjunctivitis. Bacterial conjunctivitis is encountered less frequently and it is the second most common cause of infectious conjunctivitis. Allergic conjunctivitis is encountered in nearly half of the population and the findings include itching, mucoid discharge, chemosis, and eyelid edema. Long-term usage of eye drops with preservatives in a patient with conjunctival irritation and discharge points to the toxic conjunctivitis as the underlying etiology. Effective management of conjunctivitis includes timely diagnosis, appropriate differentiation of the various etiologies, and appropriate treatment.

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Section: Introductionmentioning

confidence: 99%

Conjunctivitis: A Systematic Review

Azari

Arabi

2020

JOVR

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“…Increased concentration of uroporphyrinogen in tissues and the exposure of porphyrins to UV light cause an inflammatory reaction. 15 The biological effects of oxygen radicals, mast cell-derived mediators, and anaphylatoxin, generated as a consequence of complement activation may account for the damage of the sunlight-exposed tissues. 16 This could be the explanation for the usual location of eye injuries in the interpalpebral fissure.…”

Section: Discussionmentioning

confidence: 99%

Scleral Compromise in Hereditary Porphyria Cutanea Tarda

Soria

Cremona

Saá

et al. 2021

Journal of Current Ophthalmology

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Purpose: To report a case of bilateral scleral compromise in a male patient with hereditary porphyria cutanea tarda (PCT). Methods: Case report. Results: A 57-year-old male was referred to the Cornea Service at Hospital de Clinicas in Buenos Aires for bilateral scleral thinning. He claimed ocular discomfort and photophobia. Slit-lamp biomicroscopy revealed an oval area of deep scleral thinning without uveal prolapse, adjacent to a conjunctival hyperemic zone in the interpalpebral area, 2 mm temporal to the limbus in the right eye. The left eye presented with a conjunctivalized scleral thinning in the interpalpebral area, 2 mm temporal to the limbus. Physical examination revealed facial hyperpigmentation and hypertrichosis and multiple hypopigmented scars in hands and nails. His family history was positive for PCT. The diagnosis was made by urine porphyrin test and genetic molecular testing. In an attempt to reduce ocular and systemic levels of porphyrins, the patient was treated with oral hydroxychloroquine and repeated phlebotomies, altogether with specially designed glasses to avoid local exposure to sunlight. Conclusions: Scleral involvement is a rare manifestation of PCT. An adequate treatment, including interdisciplinary management may ameliorate ocular signs and symptoms.

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“…Ainsi, en présence de nécrose sclérale pré-perforante, des greffes de membrane amniotique peuvent être proposées, avec toutefois un risque de récidive de nécrose sur le greffon. Un patch scléral s'avère parfois indispensable afin de restaurer l'intégrité du globe en cas de perforation sclérale avérée (5). Certains auteurs proposent des greffes allogéniques limbiques cornéo-sclérales, permettant la prolifération et la migration des cellules souches limbiques, ayant en outre un effet immunosuppresseur protégeant le greffon des réactions immunologiques (8).…”

Section: Cas Cliniqueunclassified