PUK (Peripheral Ulcerative Keratitis): We’re Not in Kansas Anymore

Have you ever watched the American animated TV show Family Guy, known as much for its trademark cutaway gags as it is for its wanton vulgarity and irreverent humor? There’s one such cutaway that springs to mind, a riff on the well-known classic film the Wizard of Oz. The characters are assembled in the Emerald City where they receive their gifts from the eponymous wizard.

What was Dorothy’s, according to Family Guy’s version of the tale? Herpes — yes, herpes — which she immediately interjects about stating that “she doesn’t want herpes.” The wizard then nonchalantly informs her that “well, you’ve got it.”

Now we’re not regaling you with this little cartoon snippet as a reminder to always maintain a warm, communicative and empathetic bedside manner with your patients (unlike our good wizard). We’re making the point that nobody really wants to get herpes, not just because it is a socially stigmatized sexually transmitted disease, but also because it has a number of potential comorbidities and can lead to the development of other conditions. In rare cases, one of these is peripheral ulcerative keratitis (PUK), and we learned more about this disease during a presentation during the All India Ophthalmology Society (AIOS) International Ophthalmic Conclave (IOC) 2022.

To Time to Pucker Up with PUK

This was thanks to Update in the Management of Peripheral Ulcerative Keratitis by Dr. Somasheila Murthy, the head of service and cornea consultant at the L.V. Prasad Eye Institute (Hyderabad, India). It began with the basics, explaining that PUK is a rare inflammatory condition that can be associated with systemic autoimmune diseases and has a 50% association rate with vascular disease. Regarding its clinical features, it can present unilaterally or bilaterally, classically as a crescentic perilimbal corneal ulcer, and a subepithelial infiltrate can be observed. One is also likely to observe progressive spreading and thinning and there may also be contiguous involvement of conjunctiva, episclera and sclera, and occasional anterior chamber reaction.

Dr. Murthy was at pains to point out that the key to effectively managing PUK is to understand that its causes are twofold: local and systematic. Among local causes, one can find infections including bacterial, fungal, viral and parasitic. Bacterial sources are among the most common, with conditions like staphylococcus streptococcus and gonococcus, as are viral causes like herpes simplex and herpes zoster (we’re not sure which variant affected Dorothy). 

The picture becomes even more interesting when we examine the non-infectious local causes which are pretty varied. Traumatic chemical, thermal or radiation burns are confirmed to cause PUK and a number of factors that damage eyelids and eyelashes like entropion, cicatricial, incomplete blink and more are among other contributing factors. Neurogenic issues and autoimmune issues like Mooren’s ulcer, are among other non-trauma-based factors.

Malignancy and Management

When it comes to systemic issues the cases are broader, with immune-mediated inflammatory diseases like rheumatoid arthritis and Wegener’s granulomatosis among some of the conditions one can encounter. Dermatological issues like cicatricial pemphigoid are also sometimes found behind PUK, and one can also develop the condition after suffering from inflammatory bowel disease, “malignancy” and other systemic conditions.

Dr. Murthy’s research on the underlying conditions that cause PUK is thus comprehensive and pretty fascinating, but given the presentation including the term “management” in its title, what can we say about managing the disease? Well, Dr. Murthy didn’t disappoint here either and she provided some actionable insight into how to properly manage this difficult disease. Firstly, and this should come as no surprise, she stressed the importance of differentiating etiology.

When it came to local causes, both infectious and otherwise. Dr. Murthy recommended using topical intensive steroids and oral steroids where appropriate, and immunomodulators in younger patients or those affected unilaterally. Specifically for Moreen’s ulcer-originated PUK, she recommended the use of conjunctival resections with glue application. Prior to surgery, she also recommended providing intravenous pulse methylprednisolone or cyclophosphamide.

On the other hand, when dealing with the systemic PUK, Dr. Murthy recommended aggressive management with systemic immunosuppression and a follow-up with a rheumatologist. Treatment should also consider whether the patient is associated with scleritis. And surely, though she didn’t mention the issue specifically, a warmer bedside manner than our cartoon wizard wouldn’t hurt either.

Editor’s Note: The All India Ophthalmology Society’s 2nd International Ophthalmic Conclave (AIOS IOC 2022) was held virtually from February 18-20, 2022. Reporting for this story took place during the event.

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