Objective. To report a patient with a rare clinical presentation of margina
l keratitis and pyoderma gangrenosum (PG) that responded to the steroid-spa
ring agent azathioprine. Design. Interventional case report. Methods. Case
report and MEDLINE review of the medical literature on PG-associated periph
eral ulcerative keratitis (PUK) and its treatment. Results. A patient with
a biopsy-proven history of PG developed an explosive episode of PUK. The oc
ular inflammation was minimally responsive to topical therapy and required
significant long-term oral steroids for control. The patient's active skin
lesions and marginal keratitis responded dramatically to systemic oral azat
hioprine. Since the initiation of treatment with azathioprine, there have b
een no subsequent recurrences of PG or marginal keratitis. Conclusions. PUK
may be associated with other inflammatory conditions, such as PG and arthr
itis. Systemic azathioprine may successfully control ocular and extraocular
conditions and should be considered in the clinical setting of topical the
rapy failure and/or extraocular inflammatory conditions.