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PORPHYRIA FACTS

OCULAR MANIFESTATIONS
PART 2


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41. Do retinal hemorrhages occur in HCP?

In HCP there can be retinal hemorrhages.

SOURCE:
Psorias, Ichthyosis and Porphyria
Griffith Steiner, Robert Arffa



Retinal hemorrhages have been indicated in some porphyria patients. SOURCE:
Robert Johnson M.D.



In AIP there may be retinal hemorrhages. SOURCE:
Psorias, Ichthyosis and Porphyria
Griffith Steiner, Robert Arffa



42. What are the ocular manifestations of EPP?

Ocular manifestations of EPP include blepharitis, cicatricial ectropion, and conjunctivitis.
Lagophthalmos is a major cause of light-induced ocular surface aggravation.

SOURCE:
Christen M Mowad, MD,
Assistant Professor,
Department of Dermatology,
Geisinger Medical Center,
Pennsylvania State College of Medicine



43. What kinds of optical problems are found in AIP patients?

In AIP there is optic neuritis, cranial nerve palsy, and retinal hemorrhages.VV SOURCE:
Psoriasis, Ichthyosis, and Porphyria
Drs. Griffith Steiner, & Robert Arffa



Ptosis can occur in porphyria patients.

SOURCE:
Reynoza Sepulvada M.D.



44 What are the ocular complications found in porphyria?

Neuro-ophthalmological complications include optic neuritis, optic atrophy, ptosis, cranial nerve palsies, and retinal hemorrhages.

SOURCE:
"Psoriasis, ichthyosis, and porphyria"
Steiner, G. and R. C. Arffa (1997).
"Int Ophthalmol Clin 37(2): 41-61.



Cranial nerve palsies are part of the neuro-ophthalmological complications that can exacerbate in porphyria patients.

SOURCE:
Reynoza Sepulvada MD



45. Can ocular inflammation lead to problems in porphyria?

Inflammation can lead to vesicle and bulla formation, scarring,hyperpigmentation and superinfection.

SOURCE:
Psorias, Ichthyosis and Porphyria
Griffith Steiner, Robert Arffa



Severe scarring and hyperpigmentation can occur with ocular inflammation.
Madelyn Westergaard, PA
Ophtamology



Bulla formation can occur if ocular inflammation develops in porphyria.

Russell Metcalf
Ophtamological Services



46. Can beoplastic lesions occur with the eyes in porphyria?

Beoplastic lesions have been observed.

SOURCE:
Psorias, Ichthyosis and Porphyria
Griffith Steiner, Robert Arffa



Beoplastic lesions have been indicated in some porphyria patients.

SOURCE:
Robert Johnson M.D.
Internal Medicine



47. Can canalicular stenosis occur?

Cacatricial ectropion and punctal and canalicular stenosis can occur.

SOURCE:
Psorias, Ichthyosis and Porphyria
Griffith Steiner, Robert Arffa



Canalicular stenosis has been indicated in some porphyria patients.

SOURCE:
Robert Johnson M.D.



48. Can there be retinal bleeding in association with porphyria?

Retinal hemorrhages can occur. SOURCE:
Psorias, Ichthyosis and Porphyria
Griffith Steiner, Robert Arffa



Retinal hemorrhages can be present in porphyria patients.
Bleeding can occur from tears in the eye.

SOURCE:
Madelyn Westergaard PA
Opthamology



49. Are retinal hemorrhages associated with AIP?

In AIP there is optic neuritis, cranial nerve palsy, and retinal hemorrhages.

SOURCE:
Psoriasis, Ichthyosis, and Porphyria
Drs. Griffith Steiner, & Robert Arffa



50. Can eyelid bullae occur in HCP?

In HCP eyelid bullae can present.

SOURCE:
Psorias, Ichthyosis and Porphyria
Griffith Steiner, Robert Arffa



51. Can the eyes be scarred ?

Corneal scarring can be severe in CEP.

SOURCE:
The Porphyrias
The Less Common Porphyrias
Endocrine and Metabolic Disorders
Merck Manual of Diagnosis



Corneal scarring has been indicated in CEP patients.

SOURCE:
Reginald Gregerson
Opthamology Division



52. Does scarring occur on the eyelids in PCT?

In PCT vesicles and bullae are most often present on the eyelids.
Eyelids scarring is often found."

SOURCE:
Psoriasis, Ichthyosis, and Porphyria
Drs. Griffith Steiner, & Robert Arffa



PCT patients often have presented with bullae on the eyelids.

SOURCE:
Robert Johnson M.D.



53. Can ocular inflammation lead to problems in porphyria?

Inflammation can lead to vesicle and bulla formation, scarring, hyperpigmentation and superinfection.

SOURCE:
Psorias, Ichthyosis and Porphyria
Griffith Steiner, Robert Arffa



Superinfection can manifest in porphyria patients with ocular inflammation.

SOURCE:
Robert Johnson M.D.



Hyperpigementaiton will often occur when there is ocular inflammation in porphyria patients.

SOURCE:
Maxine DeSautel MSN RN
Optical Services United Medical Systems



54. Is there conjunctival scarring in VP?

In VP there can be conjunctival scarring.
SOURCE:
Psorias, Ichthyosis and Porphyria
Griffith Steiner, Robert Arffa



55. What types of ocular complications can occur in association with porphyria?

Neuro-ophthalmological complications include optic neuritis, optic atrophy, ptosis, cranial nerve palsies, and reinal hemorrhages.

SOURCE:
Psorias, Ichthyosis and Porphyria
Griffith Steiner, Robert Arffa



Nerve or Neuro-ophthalmological complications include optic neuritis, optic atrophy, ptosis, cranial nerve palsies, and reinal hemorrhagesalsies and retinal hemorrhages occur with neuro-ophthalmological complications.

SOURCE:
Marlene DeSautel MSN RN
Optical Services - United Medical Systems



56. Is optic nerve pallor a severe symptom?

Optic nerve pallor is believed to be secondary to anemia.

SOURCE:
Psorias, Ichthyosis and Porphyria
Griffith Steiner, Robert Arffa



Optic nerve pallor has been indicated in some porphyria patients.

SOURCE:
Marlene DeSautel MS RN
Opitcal Services- United Medical Systems



57. Is ocular disease associated with porphyria a serious condition?

As with skin disease, prevention of ocular disease is a primary concern in the porphyrias.

SOURCE:
"Psoriasis, ichthyosis, and porphyria"
Steiner, G. and R. C. Arffa (1997).
"Int Ophthalmol Clin 37(2): 41-61.



58. What is the role of photosensivity and ocular problems in porphyria?

Photosensitization can affect the eyelids, conjunctiva, cornea, sclera and, possibly, the retina.

SOURCE:
"Psoriasis, ichthyosis, and porphyria"
Steiner, G. and R. C. Arffa (1997).
"Int Ophthalmol Clin 37(2): 41-61.



Sclera has been noted in porphyria patients.

SOURCE:
Robert Johnson M.D.



59> Does canalicular stenosis occur in porphyria?

Canalicular stenosis can occur in porphyria patients.

SOURCE:
"Psoriasis, ichthyosis, and porphyria"
Steiner, G. and R. C. Arffa (1997).
"Int Ophthalmol Clin 37(2): 41-61.



60. Are there special ocular concerns with PCT?

Diffuse purple-red "heliotropic" changes of the central face, especially the periorbital skin, may be seen in PCT.

SOURCE:
"Psoriasis, ichthyosis, and porphyria"
Steiner, G. and R. C. Arffa (1997).
"Int Ophthalmol Clin 37(2): 41-61.



61. Can the sun affect the eyes and eyelids in porphyria?

Photosensitization can affect the eyelids, conjunctiva, cornea, sclera and the retina

SOURCE:
Psorias, Ichthyosis and Porphyria
Griffith Steiner, Robert Arffa



62. How does one prevent ocular problems with porphyria?

UV-blocking sunglasses are important during sun exposure.

SOURCE:
"Psoriasis, ichthyosis, and porphyria"
Steiner, G. and R. C. Arffa (1997).
"Int Ophthalmol Clin 37(2): 41-61.



63. May oral steroids be used in ocular treatment?

Oral steroids and scleral grafting may be necessary for scleritis.

SOURCE:
"Psoriasis, ichthyosis, and porphyria"
Steiner, G. and R. C. Arffa (1997).
"Int Ophthalmol Clin 37(2): 41-61.



64. Can eyes become scarred in EPP?

In Erythropoietic Protoporphyria bilateral corneal scarring may occur, with eventual blindness.
The risk for malignant conjunctival degeneration is low.

SOURCE:
Christen M Mowad, MD,
Assistant Professor,
Department of Dermatology,
Geisinger Medical Center,
Pennsylvania State College of Medicine



65. Is photophobia associated with porphyria?

Blepharospasm, lacrimation, and photophobia often are present.

SOURCE:
Psorias, Ichthyosis and Porphyria
Griffith Steiner, Robert Arffa



Photophobia can occur in the porphyrias.

SOURCE:
Robert Johnson M.D.



66. Can sunglasses help with optic damage in porphyria?

UV-blocking sunglasses are important during sun exposure to protect against optic damage in porphyria.

SOURCE:
Psorias, Ichthyosis and Porphyria
Griffith Steiner, Robert Arffa



67. Branch "vein" occlusion, segmental optic atrophy, and soft exudate are the most common ocular manifestation in acute intermittent porphyria patients. SOURCE:
"Retinal branch vessel occlusion in acute
intermittent porphyria."
Miller, S. A. and G. H. Bresnick
Annals of Ophthalmology
11(9): 1379-83. 1979



68. Are cortical cataracts found in association with porphyria? Ocular Manifestations Axial, stellate, anterior cortical cataracts,
which can be unilateral, are seen infrequently

SOURCE:
"Psoriasis, ichthyosis, and porphyria"
Steiner, G. and R. C. Arffa (1997).
"Int Ophthalmol Clin 37(2): 41-61.



69. Can AIP cause a visual field defect? A permanent unilateral visual field defect may occur due to acute intermittent porphyria.

SOURCE:
"Optic atrophy in acute intermittent porphyria."
DeFrancisco, M., P. J. Savino, et al.
American Journal of Ophthalmology
87(2): 221-4. 1979



70. How do ocular manifestations present in AIP? Often AIP patients present with retinal branch vessel occlusion and a
constellation of bizarre symptoms that might include hypertension, abdominal pain, acute psychotic behavior and/or cutaneous photosensitivity.

SOURCE:
Retinal branch vessel occlusion in acute intermittent porphyria."
Miller, S. A. and G. H. Bresnick
Annals of Ophthalmology
11(9): 1379-83. 1979



71. Can optic nerve atrophy be reversed?:

Once it has occurred, damage from optic nerve atrophy cannot be reversed. The underlying disease must be found and treated if possible to prevent further loss.

SOURCE:
Raymond S. Douglas M.D., Ph.D.
Department of Ophthalmology
UCLA Medical Center
Los Angeles, CA



72. Is there eyelid pigmentation in VP?

In VP there can be eyelid pigementation.

SOURCE:
Psorias, Ichthyosis and Porphyria
Griffith Steiner, Robert Arffa



73. Does hypertrichosis occur in HEP?

In HEP they may be hypertrichosis.

SOURCE:
Psorias, Ichthyosis and Porphyria
Griffith Steiner, Robert Arffa



74. Can muscle cramps or spasms affect the eyes?

Muscle twitching may lead to cramping and may involve the the eyelids, calf or thumb.

SOURCE:
Spasms & Muscle Contractions
Steven Angelo, M.D.
Assistant Professor of Medicine
Yale School of Medicine
New Haven, CT.



75. Where does conjunctival photosensitization occur in poprhyria?

Conjunctival photosensitization commonly manifests as hyperemia and chemosis.

SOURCE:
Psorias, Ichthyosis and Porphyria
Griffith Steiner, Robert Arffa



Chemosis can occur in porphyria due to conjunctival photosensitization.

SOURCE:
Razh Sedalenk
Visul Sciences



76. Does purpura occur in EPP?

Purpura can be seen particularly in EPP.

SOURCE:
Psorias, Ichthyosis and Porphyria
Griffith Steiner, Robert Arffa



Purpura has been clinically indicated in EPP patients.

SOURCE:
Madelyn Westergaard PA
Ophthamology



77. Can "seeing things that aren't there" be associated with porphyria?

Visual hallucinations can be seen.

SOURCE:
Psorias, Ichthyosis and Porphyria
Griffith Steiner, Robert Arffa



78. Can beoplastic lesions occur with the eyes in porphyria?

Beoplastic lesions have been observed.

SOURCE:
Psorias, Ichthyosis and Porphyria
Griffith Steiner, Robert Arffa



Bioplastic lesions occur in porphyria patients.

SOURCE:
Pavil Lukaski M.D.
Ophthamology Division



79. Is photophobia associated with porphyria and affect the eyes in porphyria patients?

Photophobia is often present in porphyria patients.

SOURCE:
"Psoriasis, ichthyosis, and porphyria"
Steiner, G. and R. C. Arffa (1997).
"Int Ophthalmol Clin 37(2): 41-61.



80. Can sclera be found associated with porphyria patients?

Sclera and scleritis are not common.

The scleritis,chiefly interpalpebral, can progress to necrosis, thinning , and even perforation, especially after the fourth decade of life .
Usually the condition is slowly progressive.

SOURCE:
"Psoriasis, ichthyosis, and porphyria"
Steiner, G. and R. C. Arffa (1997).
"Int Ophthalmol Clin 37(2): 41-61



81. Does cicatricial ectropion occur in porphyria?

Cicatricial ectropion and punctal can occur .

SOURCE:
"Psoriasis, ichthyosis, and porphyria"
Steiner, G. and R. C. Arffa (1997).
"Int Ophthalmol Clin 37(2): 41-61



82. Is there atrophy of ganglion cells associated with porphyria?

The cotton-wool spots may be brown and, over a few weeks, may become dense, black patches. Atrophy of ganglion cells and the nerve fiber layer have also been described .

SOURCE:
"Psoriasis, ichthyosis, and porphyria"
Steiner, G. and R. C. Arffa (1997).
"Int Ophthalmol Clin 37(2): 41



83. What are the retina findings in porphyria patients?

Retina Retinal findings in porphyria include retinal hemorrhages, cotton-wool spots, edema, chorioretinal pigment proliferation or atrophy, and disc edema.

SOURCE:
"Psoriasis, ichthyosis, and porphyria"
Steiner, G. and R. C. Arffa (1997).
"Int Ophthalmol Clin 37(2): 41-61.



Ganglion cell atrophy has been indicated in some porphyria patients.

SOURCE:
Robert Johnson M.D.
Internal Medicine



Color-wool spots can appear in some porphyria patients.

SOURCE:
Madelyn Westergaard PA
Ophthamology



84. Can there be problems with eyelids in porphyria?

Eyelids Photosensitization of the eyelid skin can lead to erythema and vesicle or bulla formation , which in turn can lead to scarring, hyperpigmentation, hypertrichosis, and superinfection.

SOURCE:
"Psoriasis, ichthyosis, and porphyria"
Steiner, G. and R. C. Arffa (1997).
"Int Ophthalmol Clin 37(2): 41-61



Bulla formation can occur on the eyelids.

SOURCE:
Madelyn Westergaard PA
Ophthamology



Erythema and vesicle formation can occur due to eyelid photosenitization.

SOURCE:
Robert Johnson MD
Internal Medicine



85. Can lysis provide intervention?

Early lysis of conjunctival adhesions may prevent symblepharon.

SOURCE:
"Psoriasis, ichthyosis, and porphyria"
Steiner, G. and R. C. Arffa (1997).
"Int Ophthalmol Clin 37(2): 41-61.



Lysis may be beneficial when conjunctival adhesions appear.

SOURCE:
Madelyn Westergaard PA
Ophthamology



86. Does blepharospasm affect the eyes in association with porphyria

Blepharospasm can be present in porphyria.

SOURCE:
"Psoriasis, ichthyosis, and porphyria"
Steiner, G. and R. C. Arffa (1997).
"Int Ophthalmol Clin 37(2): 41-61.



Blepharospasm has been indicated in porphyria patients.

SOURCE:
Madeklyn Westegaard
Ophthamology Division



87. How does scleritis in porphyria patient progress?

The scleritis,chiefly interpalpebral, can progress to necrosis, thinning , and even perforation, especially after the fourth decade of life .

Usually the condition is slowly progressive.

SOURCE:
"Psoriasis, ichthyosis, and porphyria"
Steiner, G. and R. C. Arffa (1997).
"Int Ophthalmol Clin 37(2): 41-61



88. In what types of porphyria can purpura be found?

Purpura has been indicated in several forms of porphyria, but most often in EPP.

Sybil Lippincott, FNP
Ophphamology Research



Purpura can also be seen, particularly in EPP.

SOURCE:
"Psoriasis, ichthyosis, and porphyria"
Steiner, G. and R. C. Arffa (1997).
"Int Ophthalmol Clin 37(2): 41-61.



In EPP patients purpura is often present.

SOURCE:
Madelyn Westegaard
Ophthamology Divison



89. Does chemosis occur in porphyria as a part of ocular manifestations?

Conjunctiva Conjunctival photosensitization commonly is manifest as hyperemia and chemosis.

SOURCE:
"Psoriasis, ichthyosis, and porphyria"
Steiner, G. and R. C. Arffa (1997).
"Int Ophthalmol Clin 37(2): 41-61



90. Does hyperemia occur in porphyria as a part of ocular manifestations?

Conjunctiva Conjunctival photosensitization commonly is manifest as hyperemia.

SOURCE:
"Psoriasis, ichthyosis, and porphyria"
Steiner, G. and R. C. Arffa (1997).
"Int Ophthalmol Clin 37(2): 41-61



91. Does hyperemia ocur in porphyria?

Hyperemia has been clinically indicated in porphyria patients.

SOURCE:
Madelyn Westergaard PA
Ophthalmoogy Division



92. Can corneal ulceration occur in association with porphyria?

: Corneal ulceration can occur in association with porphyria.

SOURCE:
"Psoriasis, ichthyosis, and porphyria"
Steiner, G. and R. C. Arffa (1997).
"Int Ophthalmol Clin 37(2): 41-61.



93. Can opacification occur in association with porphyria?

Opacification can occur. in association with porphyria in patients.

SOURCE:
"Psoriasis, ichthyosis, and porphyria"
Steiner, G. and R. C. Arffa (1997).
"Int Ophthalmol Clin 37(2): 41-61



Opacification has been indicated in many porphyria patients.

SOURCE:
Madelyn Westergaard PA



94. Is conjunctiva found in porphyria patients?

In EPP, the conjunctiva may develop a yellowish hue in the interpalpebral fissure.

SOURCE:
"Psoriasis, ichthyosis, and porphyria"
Steiner, G. and R. C. Arffa (1997).
"Int Ophthalmol Clin 37(2): 41-61.



95. Does conjunctive cause discoloration?

Discoloration in the interpalpebral tissue often ocurs in porphyria patients experiencing conjunctiva.

SOURCE:
Richar Ferrero
Ophthamology DIvision



96. What is the manifestation of the necrotic process in porphyria patients?

The necrotic process can extend across the limbus, resulting in corneal thinning and opacification and, in some cases, perforation .

More commonly, corneal complications are secondary to cicatricial ectropion.

SOURCE:
"Psoriasis, ichthyosis, and porphyria"
Steiner, G. and R. C. Arffa (1997).
"Int Ophthalmol Clin 37(2): 41-61.



97. Can there be ocular crystal formation in association with porphyria?

Crystal formation Bowman's layer and deep stromal lamellar opacification have been reported.

SOURCE:
"Psoriasis, ichthyosis, and porphyria"
Steiner, G. and R. C. Arffa (1997).
"Int Ophthalmol Clin 37(2): 41-61.



98. What are the ocular manifestations of CEP? Ocular manifestations of EP include blepharitis, cicatricial ectropion, and conjunctivitis.

Lagophthalmos is a major cause of light-induced ocular surface aggravation.

Scleral findings include interpalpebral fissures and pink fluorescence of the perilimbal sclera under a Wood light.

Blindness can occur.

SOURCE:
Erythropoietic Porphyria
Jeanette Hebel Matthews, MD,
Department of Dermatology,
The Skin Surgery Center
eMedicine Journal, May 22 2002,
Volume 3, Number 5



99. Is the 5th cranial nerve involved with porphyria?

Fifth cranial nerve paralysis can occur in AlP, with the subsequent corneal complications of hypoesthesia.

SOURCE:
"Psoriasis, ichthyosis, and porphyria"
Steiner, G. and R. C. Arffa (1997).
"Int Ophthalmol Clin 37(2): 41-61.



100. Can the cranial nerves become paralyized in asociation with porphyria?

Paralysis of the third through seventh cranial nerves may develop.

SOURCE:
"Psoriasis, ichthyosis, and porphyria"
Steiner, G. and R. C. Arffa (1997).
"Int Ophthalmol Clin 37(2): 41-61.



101. Does cranial nerve palsy present in HCP?

In HCP there can be cranial nerve palsy.

SOURCE:
Psorias, Ichthyosis and Porphyria
Griffith Steiner, Robert Arffa



102. Can Cornea Episcleritis be found associated with porphyria patients?

Cornea Episcleritis and scleritis are not common.

The scleritis,chiefly interpalpebral, can progress to necrosis, thinning , and even perforation, especially after the fourth decade of life .

SOURCE:
Psoriasis, ichthyosis, and porphyria"
Steiner, G. and R. C. Arffa (1997).
"Int Ophthalmol Clin 37(2): 41-61.



103. Can vision be restored after you are diagnosed with optic nerve atrophy?

Vision lost to optic nerve atrophy cannot be recovered.
If the cause can be identified and controlled, further visual loss progressing to blindness may be prevented.

SOURCE:
Raymond S. Douglas M.D., Ph.D.
Department of Ophthalmology
UCLA Medical Center
Los Angeles, CA



104. What is done for corneal exposure?

Standard methods for avoiding the complications of corneal exposure are employed when ectropion occurs.

SOURCE:
"Psoriasis, ichthyosis, and porphyria"
Steiner, G. and R. C. Arffa (1997).
"Int Ophthalmol Clin 37(2): 41-61.



105. Can oral steroids be used in ocular neuropathy?


Oral steroids may also be useful for neuropathy.

SOURCE:
"Psoriasis, ichthyosis, and porphyria"
Steiner, G. and R. C. Arffa (1997).
"Int Ophthalmol Clin 37(2): 41-61



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