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Clinicopathologic Reports, Case Reports, and Small Case Series
December 2002

Nodular Scleritis in a Patient With Pyoderma Gangrenosum

Arch Ophthalmol. 2002;120(12):1763-1765. doi:

Pyoderma gangrenosum is a rare chronic reactive inflammatory disorder that involves ulceration of the skin, is commonly associated with inflammatory bowel disease and arthritis, and occurs mostly in women. The ulcerative lesions of pyoderma gangrenosum are typically at the site of minor trauma and begin as small erythematous papules progressing to tender sterile pustules with central necrosis. We report a case of nodular scleritis due to pyoderma gangrenosum and our experience excising the lesion.

Report of a Case

A 39-year-old woman complained of redness and a foreign body sensation in her right eye. She also had a large 8 × 20-cm ulcer involving her right lower leg. Her medical history is significant for pyoderma gangrenosum, hemolytic anemia, large granular lymphocytic disorder, nasopharynx papillomatosis, and occasional migratory arthralgias. Findings from slitlamp examination revealed a large erythematous, raised scleral nodule adjacent to the temporal limbus of the right eye (Figure 1). The nodule was tender and had an irregular surface similar to a papilloma.

Figure 1.
Initial slitlamp photograph of
a scleral nodule adjacent to the limbus of the right eye. The yellow, elevated
lesion with prominent vessels is surrounded by focal conjunctival hyperemia.

Initial slitlamp photograph of a scleral nodule adjacent to the limbus of the right eye. The yellow, elevated lesion with prominent vessels is surrounded by focal conjunctival hyperemia.

The nodular scleritis appeared while the patient was receiving intravenous cyclosporine and methylprednisolone for the pyoderma gangrenosum ulcer of her leg. Because of the papilloma-like appearance of the scleral nodule, she was treated with topical cidofovir in addition to topical cyclosporine and prednisolone acetate. After poor response to 3 weeks of medical treatment, a diagnostic/therapeutic excisional biopsy was performed. Bacterial, viral, and fungal cultures from the biopsy specimen were all negative for organisms. The specimen showed an inflammatory cellular infiltration of the tissue diagnostic of diffuse neutrophilic mucositis, consistent with pyoderma gangrenosum (Figure 2 and Figure 3). The nodular scleritis resolved after the excisional biopsy. Topical corticosteroid was tapered and its use discontinued over a 2-month period. Therapy with systemic corticosteroids and cyclosporine were continued for the treatment of the leg ulcer, which remained stable during therapy without complete resolution. No new skin lesions appeared during this period. She had no evidence of recurrence of the scleritis during the following 7 months (Figure 4).

Figure 2.
Microscopic section of the scleral
nodule showing submucosal connective tissue with inflammatory cells that consisted
mostly of neutrophils (white arrow). A vessel is seen (black arrow) (hematoxylin-eosin,
original magnification ×20).

Microscopic section of the scleral nodule showing submucosal connective tissue with inflammatory cells that consisted mostly of neutrophils (white arrow). A vessel is seen (black arrow) (hematoxylin-eosin, original magnification ×20).

Figure 3.
High-magnification microscopic
section showing diffused neutrophilic infiltration consistent with pyoderma
gangrenosum (white arrow). A vessel is noted (black arrow). No abscess or
infectious agent is observed (hematoxylin-eosin, original magnification ×40).

High-magnification microscopic section showing diffused neutrophilic infiltration consistent with pyoderma gangrenosum (white arrow). A vessel is noted (black arrow). No abscess or infectious agent is observed (hematoxylin-eosin, original magnification ×40).

Figure 4.
Slitlamp photograph of the same
area of conjunctiva seen in Figure 1, 3 months after excisional biopsy. The
conjunctiva is flat with no ulceration or chemosis, and only minimal hyperemia.

Slitlamp photograph of the same area of conjunctiva seen in Figure 1, 3 months after excisional biopsy. The conjunctiva is flat with no ulceration or chemosis, and only minimal hyperemia.

Comment

Pyoderma gangrenosum is an autoimmune condition, resulting in painful skin ulceration after minor trauma. It is commonly associated with inflammatory bowel disease and arthritis, and approximately 10% of patients may have an IgA monoclonal gammopathy.1 Malignancies such as leukemia and lymphoma have also been associated with pyoderma gangrenosum.

The results of skin biopsies have led to formation or enlargement of ulcers.2 Because of this risk, the nodular scleritis was initially treated with immunosuppressants. Owing to the lack of response to medical treatment, an excisional biopsy of the scleral nodule was performed that did not result in additional inflammation or ulceration but rather in the resolution of the scleritis, possibly because of the preoperative and postoperative treatments with cyclosporine and corticosteroids. Of the 4 cases of pyoderma gangrenosum–related ulcerative keratitis and scleritis we reviewed,35 only 1 required biopsy, which did not result in ulceration.5

Biopsy specimens and tissue cultures are important in excluding other conditions that can mimic pyoderma gangrenosum, such as an infected ulcer. In this current case report, histopathologic findings of inflammatory cell infiltrate with or without abscess formation is consistent with, but not specific to, pyoderma gangrenosum.2

Patients with ocular involvement of pyoderma gangrenosum have responded to a variety of treatments. Three reported cases of ulcerative keratitis have been treated successfully with oral and topical coritcosteroids, oral cyclophosphamide, and oral azathioprine.3,4 A case of scleral ulceration responded to cryotherapy and systemic cortisone.5 Our case of nodular scleritis resolved with a combination of an excisional biopsy with adjunctive systemic and topical immunosuppressants.

Corresponding author and reprints: Christopher N. Ta, MD, Department of Ophthalmology, Stanford University, 900 Blake Wilbur Dr, Room W3002, Stanford, CA 94304 (e-mail: cta@stanford.edu).

References
1.
Powell  FCSchroeter  ALSu  WPPerry  HO Pyoderma gangrenosum and monoclonal gammopathy. Arch Dermatol. 1983;119468- 472Article
2.
Powell  FCollins  S Pyoderma gangrenosum. Clin Dermatol. 2000;18283- 293Article
3.
Bishop  PTullo  A Pyoderma gangrenosum and necrotizing sclerokeratitis. Cornea. 1998;17346- 347
4.
Brown  BAParker  CTBower  KS Effective steroid-sparing treatment for peripheral ulcerative keratitis and pyoderma gangrenosum. Cornea. 2001;20117- 118Article
5.
Happle  RSchiffer  HKovary  P Ocular involvement in pyoderma gangrenosum. Arch Dermatol. 1977;1131612Article
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