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Clinical Ophthalmology 2008:2(1) 203–206
C A S E R E P O RT
Ligneous conjunctivitis in a patient with Crohn’s
José M Herreras1
M del C Méndez2
1Ocular Immunology and Uveitis
Unit, Institute of Ophthalmobiology
(IOBA), University of Valladolid,
Valladolid, Spain and Hospital
Universitario de Valladolid, Spain;
2Ocular Pathology and Lab Registry
“Miguel N. Burnier”, Institute of
Ophthalmobiology (IOBA), University
of Valladolid, Valladolid, Spain
Correspondence: José M. Herreras
IOBA, Facultad de Medicina, Ramón y
Cajal 7, Valladolid E-47005, Spain
Tel +34 983 184750
Fax +34 983 423235
Objective: Report the case of a patient with Crohn´s disease and ligneous conjunctivitis.
Methods: Interventional case report of a 27-year old female patient, with a 4-year history
of Crohn´s disease, was seen for right eye conjunctival ulcer after resolved chemical trauma.
Conjunctival biopsy was performed for histopathology, immunofl uorescence microscopy, and
Results: Microbiology tests were negative for bacteria and fungi. PCR was negative for atypical
mycobacteria and Herpes. Ocular Crohn´s disease, Whipple disease, and amyloidosis were ruled
out. Immunofl uorescence microscopy revealed characteristic IgG κ and λ light chain deposits,
and fi brin deposition was confi rmed through Lendrum´s Martius, Scarlet, and Blue technique.
Endogenous plasminogen levels were normal. Recurrent ulcers did not resolve after treatment
with infl iximab, but only after four surgeries, topical steroids, 1% cyclosporine, heparin (5000
units/ml), and hyaluronidase (1.5 mg/ml). After 9 months of follow-up, nasal symblepharon
was observed as sequela.
Conclusions: We report the case of a patient with unilateral ligneous conjunctivitis, triggered
by chemical injury, and Crohn´s disease.
Keywords: Crohn´s disease, immunofl uorescence microscopy, ligneous conjunctivitis, plas-
minogen, ulcerative conjunctivitis
Ligneous conjunctivitis (LC) is a rare form of chronic conjunctivitis characterized by
the development of fi brin-rich, woody-like pseudomembraneous tissue in the tarsal
conjunctiva (Schuster and Seregard 2003). It occurs in association with external irri-
tants and ocular surgery (Schuster and Seregard 2003).
Plasminogen defi ciency has recently been found to be the most common cause of
LC, and the lesions are the result of an impaired wound healing response (Schuster and
Seregard 2003). LC seems to be the most common symptom of this systemic disorder,
since it usually occurs with other mucosal involvement (Schuster and Seregard 2003).
Crohn´s disease (CD) is an infl ammatory bowel disease with ocular involvement
as an extraintestinal manifestation. The eye disease commonly presents as anterior
uveitis and, rarely, with naevus and conjunctival or lid ulcers (Lendrum et al 1962;
Schuettenberg 1991; Hegab and al-Mutawa 1994; Zoli et al 1997; Diaz-Valle et al
2004). Based on clinical evolution and histopathology fi ndings, we report a rare case
of a patient with CD and LC triggered by chemical injury.
A 27-year-old female patient presented at our center with a 6-week history of right eye
pain and ulceration. The symptoms appeared one month after a resolved conjunctival
chemical burn with 17% hydrochloric acid. The recurrent corneal and conjunctival
Clinical Ophthalmology 2008:2(1)
Fuentes-Páez et al
ulcers progressed despite topical corticosteroids, 1%,
cyclosporine A (CsA), antibiotics, and artifi cial tears.
The patient also had a 4-year history of aggressive CD,
confi rmed by intestinal biopsy, which required immuno-
suppressive therapy with azathioprine, anti-TNF-alpha
treatment, and intestinal resection. Her CD had been stable
for last 6 months.
Visual acuity was 20/200 for her right eye and 20/20 for
the left eye. Our initial slit-lamp examination of her right
eye revealed plaque-like ulcerations in the nasal bulbar
conjunctiva and in the inferior and superior tarsal conjunctivas
(Figure 1A, B). The left eye was normal. Systemic workup
ruled out sarcoidosis, systemic vasculitis, rickettsias, brucella,
syphilis, chlamydia, Lyme disease, and HIV.
Bulbar, upper, and lower fornix conjunctival resections
were performed on the affected eye. Tissue specimens from
the resection were processed for special stains including
Gram and acridine orange for bacteria, calcofl uor and peri-
odic acid Schiff (PAS) stains for fungi and/or Whipple dis-
ease, and Congo red for amyloidosis. The presence of atypical
mycobacteria and Herpes were determined by polymerase
chain reaction (BIOTOOLS B and M Labs, Madrid, Spain).
All of the tests were negative.
Tissue sections for routine histopathological examination
were stained with hematoxylin and eosin (H&E) and PAS stains.
Fibrin deposits were assessed by Lendrum´s Martius, Scarlet,
and Bluetechnique (MSB) technique (Lendrum et al 1962).
Immunofl uorescence microscopy for IgG and κ, λ light
chain deposits was performed with polyclonal antihuman
antibodies from BioGenex Laboratories (San Ramon,
CA, USA) and DAKO Corp. (Carpintería, CA, USA),
respectively. The secondary antibody was FITC-conjugated
anti-rabbit IgG (Sigma, St. Louis, MO, USA).
Histopathology reported surface ulcerations with
an underlying thick band of hyaline-like eosinophilic
material, granulation tissue, and lymphocytic infi ltration
(Figure 2A). Abundant fi brin deposition was confi rmed
with the MSB technique (Figure 2B). No granulomas were
found. Histopathology and IF fi ndings were compatible
with LC. Her systemic plasminogen values were tested and
“verbally reported” within normal limits (0.7–1.0 U/ml), on
two separate occasions.
After the conjunctival resection, her symptoms and signs
resolved, but then recurred two weeks later (Figure 1C). Her
gastroenterologist indicated treatment with 3 doses of infl ix-
imab (5 mg/kg/day, Remicade, Schering-Plough, Kenilworth,
NJ), but ocular signs did not improve. A second surgical
excision was performed and treatment with 1% CsA three
times a day (tid) initiated.
Three weeks later, a third excision was performed, since
the lesion recurred, became very painful, and progressed
toward the inferior palpebral skin. Otorrhinolaryngology
evaluation was solicited and reported as normal.
Topical heparin (5000 units/ml) tid and fl uorometho-
lone tid was added. Due to further progression, a fourth
excision was performed one month later, and topical
hyaluronidase (1.5 mg/ml) tid added. Six months after
treatment, the lesions resolved but an inferior nasal sym-
blepharon developed as sequela, after a 9-month follow-up.
Figure 1 Initial right eye slit-lamp examination. The nasal bulbar and inferior conjunctivas (A) and the superior tarsal conjunctiva (B) had plaque-like ulcerations. The initial
conjunctival ulceration was followed by a recurrence of the inferior tarsal ulcer (C). A nasal symblepharon remained as a sequela after a 9-month followup (D).
Clinical Ophthalmology 2008:2(1)
Ligneous conjunctivitis in a patient with Crohn’s disease
Ocular involvement, as an extraintestinal manifestations
of infl ammatory bowel disease, occurs in less than 12% of
cases (Ghanchi and Rembacken 2003). It manifests as uveitis,
episcleritis, scleritis, nonspecifi c follicular conjunctivitis, lid
marginal ulcers, during active or inactive disease (Salmon
et al 1991; Schuettenberg 1991; Hegab and al-Mutawa 1994;
Zoli et al 1997; Ghanchi and Rembacken 2003; Diaz-Valle
et al 2004). She had been asymptomatic for over one year
with no treatment and remained as such throughout her
Previously reported cases of lid margin and conjunctival
ulcers in CD have resolved after systemic and topical corti-
costeroid treatment (Hegab and al-Mutawa 1994; Diaz-Valle
et al 2004). We initially believed these conjunctival ulcers
were due to her CD, which is why infl iximab therapy was
administered, after consulting with her gastroenterologist,
however she did not respond. In addition, histopathology
fi ndings were more compatible with LC, since no granulomas
were reported, which could have been triggered by previous
chemical injury (Schuster and Seregard 2003).
LC is a rare disease, with a prevalence of 1.6 per million
in Europe, and due to the inheritance of an autosomal reces-
sive gene (Schuster and Seregard 2003). It involves a great
variety of systemic manifestations including periodontitis,
renal calculi, vulvovaginitis, respiratory disease, otitis media,
dermatologic lesions, and hydrocephalus, among others
(Schuster and Seregard 2003). Our patient did not report any
of these systemic manifestations other than an ocular chemi-
cal burn one month before the lesions appeared.
LC patients may have decreased levels of plasminogen,
which arrests wound healing at the granulation tissue
formation stage, and subsequently forms a fibrin-rich
pseudomembranous tissue (Schuster and Seregard 2003).
However, normal serum plasminogen levels do not exclude
LC because it is not a diagnostic criterion, but a predisposing
factor, as would be in this case.
Other triggers for the development of LC include expo-
sure to chemical irritants and conjunctival surgery (Schuster
and Seregard 2003). In this patient, exposure to chemical
irritants could have triggered the LC, which recurred after
multiple surgical interventions.
This patient’s conjunctival ulcers healed after topical
CsA, heparin, and hyaluronidase therapy, which have
been reported as effective treatments for LC (Schuster and
The fact that conjunctival cicatrizing sequela is not
characteristic of LC also challenged our initial diagnosis,
although corneal scarring has been reported as a LC sequela,
however the previous chemical burn strongly supported this
diagnosis (Hidayat and Riddle 1987).
Histopathologically, LC is characterized by subepithelial,
eosinophilic, amorphous material admixed with acute
and chronic inflammatory cells, positive PAS staining,
and negative Congo red staining (Hidayat and Riddle
1987; Holland et al 1989; Klebe et al 1999). In this case
histopathology fi ndings, abundant fi brin deposition, chronic
inflammation with granulation tissue and lymphocytes,
absence of granulomas, and negative Congo red staining,
coincided with LC (Hidayat and Riddle 1987; Holland et al
1989; Klebe et al 1999; Schuster and Seregard 2003).
Given the history of her immunobiological disease,
triggering chemical burn, clinical progression despite infl iximab
therapy, characteristic histopathology, multiple recurrences,
and resolution after combined surgical and topical treatments,
LC was our fi nal diagnosis. The LC in this CD patient was
refractory to conventional treatments, however the coexistence
between both diseases can be considered as casual.
We present a rare case of LC, triggered by conjunctival
chemical injury, casually coexisting in a patient with CD.
Figure 2 Histopathology of conjunctival biopsies. The superfi cial ulceration had an underlying thick band of hyaline-like eosinophilic material, probably composed of fi brin
(arrow). Granulation tissue and lymphocytic infi ltration were apparent in H&E (A) and fi brin (arrow) was confi rmed with Lendrum staining (B). The tissue sample was
negative for Congo red.
Clinical Ophthalmology 2008:2(1) Download full-text
Fuentes-Páez et al
Dr. S Seregard of St. Erik´s Eye Hospital, Stockholm Sweden,
for performing the MSB technique for fi brin deposition
confi rmation. Graciana Fuentes-Páez was supported by a
grant from Carolina Foundation, Ministry of Foreign Affairs,
Spain. The authors have no confl ict or commercial interest.
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