The peripheral corneal melting syndrome and psoriasis:coincidence or association?

S.VARMA, A.F.WOBOSO,* C.LANE* AND P.J.A.HOLT

Department of Dermatology and *Department of Opthalmology, University Hospital of Wales, Cardiff CF4 4XW, U.K.

Accepted for publication 28 February 1999

Summary The peripheral corneal melting syndrome (PCMS) is a rare disease consisting of marginal corneal

thinning that can progress to perforation. The PCMS carries a grave prognosis and it is of vital

importance that dermatologists are aware that this may be responsible for a painful red eye in apatient with psoriasis. We highlight the features of the PCMS developing in an elderly woman with

long-standing psoriasis to increase awareness of its signi®cance, and hypothesize that an association

may exist between the two conditions. Only one previous report has been published in which theauthors speculate on the possible association of this syndrome with psoriasis. That few other cases

have been described is either a consequence of under-reporting by both ophthalmologists and

dermatologists unaware of a link or because the relationship between the syndrome and psoriasis isgenuinely coincidental.

Key words: peripheral corneal melting syndrome, psoriasis

It is not widely appreciated that approximately 10% of

psoriatic patients have some form of ocular signs.1

Psoriasis can affect the lids, conjunctiva, cornea and

anterior uveal tract.2 The ocular disease is usually

bilateral and often occurs during an exacerbation ofthe psoriasis.3 The peripheral corneal melting syndrome

(PCMS) is a rare disease consisting of marginal corneal

thinning that can progress to perforation. It has beendescribed in association with systemic vasculitis,4

connective tissue disorders such as rheumatoid arthritis

and with SjoÈgren's syndrome, polyarteritis nodosa andWegener's granulomatosis.5,6 However, it can occur in

the absence of systemic disease. Boss et al. suggestedthat the association between the PCMS and psoriasis in

two of their patients was not coincidental.7 The prog-

nosis in the PCMS is grave. It is therefore crucial fordermatologists to have a high index of suspicion when

treating a patient with psoriasis who has a sore red eye.

We report a patient with long-standing psoriasis whodeveloped the PCMS and speculate that an association

may exist between the two conditions.

Case report

An 83-year-old woman with widespread chronic plaque

psoriasis of over 20 years duration presented with a 6-week

history of photophobia, blurred vision and a painful redeye. Her past ocular history consisted of dry eye for

which she was on topical lubricants. There were no

concomitant medical problems, in particular no evi-dence of rheumatoid arthritis or collagen vascular dis-

order. She had previously had left ventricular failure,

aortic aneurysm repair and mild dementia. Her medica-tions were aspirin 75 mg once daily, enalapril 5 mg once

daily and occasional diclofenac sodium 50 mg with

misoprostol 200 mg tablets. Examination revealed thinpink psoriatic plaques affecting the upper trunk, chest,

back and hips. Ophthalmic examination by slit-lamprevealed a deep circumferential corneal ulcer at the

limbus with undermined edges in the right eye extending

to 90% of corneal thickness (Fig. 1). Corneal meltsyndrome of Mooren was diagnosed, which is a speci®c

form of corneal melting.

Systemic examination was normal. Full blood count,blood urea, electrolytes, glucose, liver function tests,

complement levels, rheumatoid factor, antinuclear anti-

bodies, antineutrophilic cytoplasmic antibodies andsyphilis serology were normal or negative. The erythro-

cyte sedimentation rate was elevated at 37 mm in the

®rst hour (normal 1±10). The psoriasis responded totreatment with topical emollients and twice-daily calci-

potriol ointment. Her ocular treatment consisted of

British Journal of Dermatology 1999; 141: 344±346.

344 q 1999 British Association of Dermatologists

Correspondence: Dr S.Varma. E-mail: varma@cf.ac.uk

intensive arti®cial eye drops, lacrilube ointment, punctal

occlusion and botulinum toxin which induced protec-tive ptosis. A trial of oral prednisolone, 40 mg daily over

a 2-week period, did not improve the Mooren's corneal

melt. At the last out-patient review, the cornea was notperforated and the eye was relatively comfortable.

Discussion

Ocular signs have been reported to occur in approxi-

mately 10% of cases of psoriasis.1,3 Kaldeck reportedocular involvement in 11 of 90 non-selected patients

with psoriasis.8 As psoriasis is one of the most common

skin conditions, this association has implications fordermatologists and ophthalmologists. Ocular features of

psoriasis include inferior punctate corneal opacities,3

conjunctivitis, super®cial punctate keratitis,2 iritis,chronic iridiocyclitis,9 uveitis,10 trichiasis, symble-

pharon and dry eyes.8 In¯ammatory eye lesions werea frequent accompaniment to psoriatic arthritis, with

conjunctivitis occurring in 20% of patients in one

study.11 On the eyelid, blepharitis, seborrhoeic changes,

loss of eyelashes or the characteristic plaque may bepresent. There has been speculation that facial and

eyelid psoriasis may be a marker of severe psoriasis.12

Corneal lesions have been documented but are rareand consist of super®cial or deep opacities as well as

neovascularization, epithelial erosions and rarer, but

more important, corneal stromal melts. Corneal involve-ment usually occurs when there is active disease in the

skin of the lids.3 The PCMS is most often described in

association with rheumatoid arthritis, SjoÈgren's syn-drome, polyarteritis nodosa and Wegener's granuloma-

tosis, and can progress to ulceration.5,6 However, the

Mooren's type distinguishes itself by its intact cornealepithelium and occurrence in the absence of systemic

disease. Mooren's corneal melt is a chronic super®cial

corneal defect of unknown aetiology occurring withoutperforation in elderly people. It progresses slowly and

unrelentingly, accompanied by much pain, until the

entire cornea may become involved.4 There are threetypes: (i) unilateral Mooren's ulceration (as in our

patient); (ii) bilateral aggressive Mooren's ulceration;

and (iii) bilateral indolent Mooren's ulceration. Theulceration commences in the peripheral cornea and

may progress leaving a thin vascularized opaque

cornea. It is bilateral in 25% of cases. Symptoms includesevere pain, photophobia and blurred vision due to

corneal astigmatism. Speci®c treatments are indicateddepending on the type of ulceration. For type (i), therapy

is dif®cult and all treatments are unsatisfactory. As

corneal destruction is an autoimmune response to therelease of cornea-speci®c antigens, aggressive local or

systemic immunotherapy is indicated.4 Refractory cases

may require removal of the whole of the corneal stroma,thereby removing the source of the antigen, relieving

pain and halting the in¯ammatory process.4 Other

therapies including lamellar keratoplasty have beentried with limited success. Treatment with monoclonal

antibodies may become possible in the future. Both the

psoriasis and the corneal melting should be treatedearly and enthusiastically.

As psoriasis is so common and corneal disease so rare,

it would be reasonable to regard the link betweenpsoriasis and the PCMS as genuinely coincidental.

However, under-diagnosis is likely because of the possi-

bility that eye disease is under-recorded by dermatolo-gists and skin disease by ophthalmologists. We describe

this case to increase awareness of associations between

ocular disease and psoriasis and to hypothesize that anassociation may exist between the PCMS and psoriasis.

Because the PCMS carries a grave prognosis, it is crucial

for dermatologists to have a high index of suspicion for

CORNEAL MELTING AND PSORIASIS 345

q 1999 British Association of Dermatologists, British Journal of Dermatology, 141, 344±346

Figure 1. (a) Slit-lamp examination demonstrating Mooren's ulcera-

tion. (b) Slit-lamp examination with ¯uorescein dye showing Mooren'sulceration.

this condition in a patient with psoriasis presenting witha red eye. An urgent ophthalmology consultation

should be requested to establish the diagnosis and

institute treatment.

Acknowledgments

Part of this article was presented at the British Associa-tion of Dermatologists Annual Meeting, Brighton, U.K.,

7±11 July 1998, and appears in abstract form: Varma S,

Feyi-Waboso A, Lane C, Holt PJ. Is corneal melting anocular manifestation of psoriasis? Br J Dermatol 1998;

139 (Suppl. 51): 60±1.

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