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.
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.
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
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).
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).
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.
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,3- 5 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: email@example.com).
Braun MM, Wong IG, Ta CN. Nodular Scleritis in a Patient With Pyoderma Gangrenosum. Arch Ophthalmol. 2002;120(12):1763-1765. doi: