7. Hirose T, Tani T, Shimada T, Ishizawa K, Shimada S, Sano T.

Immuno- Can J Ophthalmol 2019;54:e283–e285

histochemical demonstration of EMA/Glut1-positive perineurial cells and
CD34-positive fibroblastic cells in peripheral nerve sheath tumors. Mod
Pathol. 2003;16:293–8.
8. Agaimy A. Microscopic intraneural perineurial cell proliferations in patients
with neurofibromatosis type 1. Ann Diagn Pathol. 2014;18:95–8.
0008-4182/17/$-see front matter © 2019 Canadian Ophthalmological
Society. Published by Elsevier Inc. All rights reserved.
https://doi.org/10.1016/j.jcjo.2019.03.014

Morgellons disease leading to corneal
perforation and enucleation
Morgellons disease is a somewhat controversial and poorly
understood skin disorder characterized by crawling sensations
associated with slow-healing, ulcerated, filamentous skin
lesions.1 Patients often have accompanying musculoskeletal
and neurological manifestations, suggested by some authors
to resemble the symptoms of Lyme disease.2,3 Although sev-
eral authors have proposed various diagnostic criteria, there is
currently no widely accepted consensus. Middelveen et al.
proposed that Morgellons disease represents a somatic Lyme
disease like illness associated with spontaneously appearing
skin lesions, the key diagnostic criterion being multicoloured
filaments protruding from or embedded in the skin, often
requiring 50 £ magnification to visualize.2 The Centers for
Disease Control and Prevention (CDC) suggested that “Mor-
gellons” is a lay term used to describe an unexplained constel-
lation of symptoms, with manifestations primarily involving
the skin.1 Their proposed definition is: reported fibers or
other solid materials protruding from the skin, and skin
lesions or disturbing skin symptoms.1
To the best of our knowledge, there is only one previ-
ous case of Morgellons disease reported in the ophthal-
mic plastic literature.4 We present a case of Morgellons
disease with significant cicatricial changes resulting in
exposure keratopathy, corneal perforation, and endoph-
thalmitis leading to enucleation.
A 63-year-old Caucasian woman presented to the emer-
gency on-call clinic with a large descemetocele, diffuse cor-
neal infiltrate, and severe cicatricial ectropion of the right
lower eyelid. She reported a 5-year history of preoccupation
with extracting “string-like tissue” due to beliefs of an occipi-
toparietal infection “creeping” to her face.
She was admitted to hospital for fortified topical antibiot-
ics; however, she developed a large corneal perforation further
complicated by endophthalmitis. As she was considered to be
a poor candidate for therapeutic penetrating keratoplasty, the
right eye was enucleated and a porous polyethylene orbital
implant was inserted. She declined concurrent repair of her
cicatricial ectropion. On histopathology, the enucleated globe
showed a poorly formed anterior chamber with evidence of a
large uveal abscess and significant inflammatory changes con-
sistent with pyogenic endophthalmitis (Fig. 1).

Fig. 1—Histopathological analysis of the enucleated globe. Specimens were stained with hematoxylin–eosin. (A) Contents in the
globe are relatively disorganized. A large uveal abscess and inflammatory changes consistent with pyogenic endophthalmitis are
present. (B) The anatomy of the anterior chamber is difficult to discern, due to the perforated cornea. (C) Significant inflammation,
indicative of acute endophthalmitis and formation of an uveal abscess, is noted.

CAN J OPHTHALMOL—VOL. 54, NO. 6, DECEMBER 2019 e285

Correspondence

Fig. 2—(A) On follow-up after enucleation, the patient presented with a 2-mm area of implant erosion and a cicatricial ectropion.
(B) Full-thickness skin graft to the lower eyelid to lengthen the anterior lamellae. (C) Multiple skin lesions over her right cheek,
upper eyelid, medial canthus, and over the nose bridge starting to affect her left medial canthus. (D) Dermis fat graft failure and
removal due to persistent excoriation behaviour. (E) Appearance of the left eye before cicatricial changes. (F) Medial left upper
eyelid retraction due to cicatricial changes 8 months after the patient’s previous surgery.

On routine postenucleation follow-up, we noted a 2-mm
area of implant erosion and significant cicatricial ectropion
(Fig. 2A) secondary to self-inflicted repeated injury to the
periocular soft tissues. Topical gatifloxacin QID and tobra-
mycin/dexamethasone BID were started; unfortunately, her
implant exposure did not heal, and 1 month later, she under-
went an attempt at repair. The implant was irrigated with
cefazolin, and a scleral patch was sewn over the area of
implant erosion and secured below the conjunctiva. She
received a full-thickness skin graft to the lower eyelid to
lengthen the anterior lamellae in order to address the ectro-
pion (Fig. 2B). A skin biopsy showed mild, nonspecific,
chronic inflammatory changes. Over the next 5 months, she
had multiple visits for a painful right orbit. There were
numerous excoriations over her right cheek, right upper eye-
lid, and left medial canthus (Fig. 2C) from persistent picking
of her orbit to remove “protruding fibers.” As a result, the
implant became re-exposed and was removed. A dermis fat
graft was inserted with the hope that it would allow for better
bio-integration. However, she continued picking at her orbit,
resulting in dermis fat graft atrophy (Fig. 2D).
Several skin biopsies were performed to investigate her
symptoms, some of which showed areas of dermal fibrosis
with focal lichenoid inflammation and features of lichen
simplex chronicus. Initially, her skin biopsy results were
thought to represent lichen planus. However, several char-
acteristic changes suggestive of lichen planus were absent,
including hypergranulosis, hyperkeratosis, and lymphohis-
tiocytic infiltrate at the dermal-epidermal junction.5 Fur-
thermore, lichen planus favours the flexor surfaces, trunk,
and oral or genital mucosa with only 6 reported cases iso-
lated to the eyelids.5 After multiple biopsies and unsuccess-
ful fibre visualization, infectious disease was consulted to
rule out underlying infectious etiologies, all of which ren-
dered negative. Additionally, results of allergy patch testing
were unremarkable and her Borrelia infection status is
unknown as she declined testing. She was subsequently
diagnosed with Morgellons disease.
Over the next 8 months, she began picking at her left upper
eyelid (Fig. 2E) and presented with cicatricial retraction, expo-
sure keratopathy, punctate epithelial erosions, and a dellen in
her left eye (Fig. 2F). Although the patient brought specimens
in a jar, the contents corresponded to an area of recently ulcer-
ated skin and the authors were unable to visualize any fibers.
Subsequently, a medial third permanent tarsorrhaphy was per-
formed as she declined eyelid retraction repair. Repeated skin
trauma resulted in stretching and distortion of the tarsorrha-
phy, causing tenting of the eyelid medially, and she also devel-
oped an 8 mm by 8 mm area over the right lateral brow with
bone exposure. Her corneal surface continued to

e286 CAN J OPHTHALMOL—VOL. 54, NO. 6, DECEMBER 2019


decompensate and she developed a 1.6 mm by 2.4 mm left
corneal ulcer. She was admitted to hospital and started on for-
tified topical cefazolin and tobramycin in conjunction with
systemic doxycycline aimed at inhibiting matrix metalloprotei-
nases to limit further collagenolysis and corneal thinning. After
an unsuccessful attempt to extend the tarsorrhaphy to the mid-
dle third, an attempt at upper and lower eyelid reconstruction
using full-thickness skin grafts was performed. The right lateral
brow wound was closed with a rotational flap.
During her most recent admission, psychiatry diagnosed
her with delusional disorder, somatic type (297.1, F22)6 and
agreed with the diagnosis of Morgellons disease. Risperidone
and quetiapine were trialled on 2 separate occasions, but dis-
continued by the patient both times. She recently started oral
paliperidone 3 mg once daily but declined ongoing psychiatry
follow-up.
Cicatricial changes most commonly arise secondary to
trauma, burns, surgery, or malignancies masquerading as
nonhealing eyelid lesions.4,7 After ruling out the above with a
proper evaluation, biopsy, culture, and subspecialty input, it
is important to consider Morgellons disease in a patient pre-
senting with cicatricial eyelid changes.
The etiology of Morgellons disease is currently unknown,
although many dermatologists and psychiatrists consider it a
subtype of delusional parasitosis.1,8,9 Laboratory tests indica-
tive of inflammation or infection are routinely normal and
skin biopsies reveal nonspecific findings.1,8 Furthermore, the
CDC could not identify any underlying medical or infectious
causes and found that fibers were consistent with superficial
skin or cotton.1 In contrast, some authors suggest that Borre-
lia spirochetal infection serves as an infectious etiology, with
evidence of infection being found on laboratory tests along
with apparent resolution of symptoms in response to antibi-
otic therapy.2,3,10 Our observations for this case are consis-
tent with a delusional etiology.
The medical management of Morgellons involves low-dose
typical and atypical antipsychotics.11 13 Although antipsy-
chotics are considered first-line treatment, a systematic review
found limited evidence for their effectiveness.13 Given the
different proposed disease processes, treatment to cover an
infectious etiology may be considered. In our opinion, anti-
microbial therapy was not indicated in our patient given that
in Alberta, Canada, only 2% of ticks submitted to Alberta
Health in 2017 were positive for Borrelia burgdorferi.14 Addi-
tionally, only 87 cases of Lyme disease were reported in
Alberta from 1991 to 2016, all of which were acquired while
travelling outside of Alberta.15 Our patient did not endorse a
recent travel history.
Surgical management of patients is often challenging
due to increased risks of wound dehiscence, postoperative
infection from excoriation,4 or, in this case, implant expo-
sure after enucleation. Ectropion repair was deferred in the
previously published case of Morgellons in consideration
of wound dehiscence and an aggressive eye rubbing his-
tory.4 For Morgellons patients with cicatricial lid changes,
it may be best to defer surgery and opt for medical
CAN J OPHTHALMOL—VOL. 54, NO. 6, DECEMBER 2019
Correspondence
management with antibiotic ointment and good lubrica-
tion if exposure keratopathy is present. A referral to psy-
chiatry is also key.11
To obtain patient consent and compliance with psychiatric
treatment, many authors advocate establishing a strong thera-
peutic alliance.8,11,12 Conducting a thorough and complete
skin and ophthalmological assessment validates patient con-
cerns, and rules out other organic causes of cicatricial lid
changes.11 It is prudent to perform a skin biopsy on all new
patients presenting with Morgellons symptoms as some
patients are more willing to consider alternative diagnoses
when confronted with a negative biopsy.8
Given its rising incidence and potentially devastating
impact, this case highlights the importance of considering
Morgellons disease in the differential diagnosis for patients
presenting with cicatricial eyelid changes.
Footnotes and Disclosure:
The authors have no proprietary or commercial interest in any mate-
rials discussed in this article.
Jingyi Ma, Kelsey A. Roelofs, Jaime Badilla
University of Alberta, Edmonton, Alberta
Correspondence to:
Jaime Badilla, MD; jbadilla@gmail.com
TAGEDH1REFERENCESTAGEDEN
1. Pearson M, Selby J, Katz K, et al. Clinical, epidemiologic, histopatho-
logic and molecular features of an unexplained dermopathy. PLoS One.
2012;7:e29908.
2. Middelveen MJ, Fesler MC, Stricker RB. History of Morgellons disease:
from delusion to definition. Clin Cosmet Invest Dermatol. 2018;11:
71–90.
3. Middelveen M, Burugu D, Poruri A, et al. Association of spirochetal
infection with Morgellons disease. F1000Res. 2013;2:25.
4. Sandhu R, Steele E. Morgellons disease presenting as an eyelid lesion.
Ophthalmic Plast Reconstr Surg. 2016;32:e85–7.
5. Huang Y, Wang C, Potenziani S, Hsu S. Lichen planus of the eye-
lids: a case report and review of the literature. Dermatol Online J.
2017;23(2).
6. American Psychiatric Association. Diagnostic and Statistical Manual of
Mental Disorders. 5th ed Arlington, VA: American Psychiatric Associa-
tion; 2013.
7. de Menezes Bedran E, Correia Pereira M, Bernardes T. Ectropion.
Semin Ophthalmol. 2010;25:59–65.
8. Robles D, Olson J, Combs H, et al. Morgellons disease and delusions of
parasitosis. Am J Clin Dermatol. 2011;12:1–6.
9. Foster AA, Hylwa SA, Bury JE, et al. Delusional infestation: clinical pre-
sentation in 147 patients seen at Mayo Clinic. J Am Acad Dermatol.
2012;67. 673.e1 e10.
10. Savely VR, Leitao MM, Stricker RB. The mystery of Morgellons disease:
infection or delusion? Am J Clin Dermatol. 2006;7:1–5.
11. Driscoll M, Rothe M, Grant-Kels J, Hale M. Delusional parasitosis: a
dermatologic, psychiatric, and pharmacologic approach. J Am Acad
Dermatol. 1993;29:1023–33.
12. Koo J, Lee C. Delusions of parasitosis: a dermatologist’s guide to diagno-
sis and treatment. Am J Clin Dermatol. 2001;2:285–90.
13. Lepping P, Russell I, Freudenmann R. Antipsychotic treatment of
primary delusional parasitosis. Br J Psychiatry. 2007;191:198–205.
e287
Correspondence
14. Government of Alberta. Tick Surveillance 2017 Summary. Edmonton,
AB: Government of Alberta; 2018.
15. Alberta Health. Lyme disease & tick surveillance in Alberta: Lyme
disease cases in Alberta table. www.health.alberta.ca/health-info/lyme-
disease.html. [accessed Oct 8 2018].
A curious case of arteritis: infectious,
inflammatory, or both
Takayasu arteritis (TA) is a rare, chronic, progressive large-vessel
granulomatous disease.1,2 The specific pattern of arterial involve-
ment in TA patients differs between geographic regions; how-
ever, the aorta and its main branches are most commonly
affected in a contiguous fashion.3,4 Ocular manifestations are
also common in TA and have been reported in up to 45% of the
affected patients in some reports.5,6 Ophthalmic presentations
can occur due to chronic ocular hypoperfusion due to stenosis of
large vessels and less commonly the central retinal artery.7 In
addition, TA patients can present with hypertensive retinopathy
and papilledema in severe cases.5,8 In this report, we describe the
ophthalmic findings of a patient with long-standing untreated
TA presenting with acute visual disturbances.
TAGEDH1CASE REPORTTAGEDEN
A 57-year-old female was acutely referred to the general eye
clinic from the emergency department for a 3-day history of a
large floater in her field of vision. The patient described the
floater as central in location and without any photopsias. Past
ocular history was otherwise unremarkable. She described her
medical history as unremarkable aside from annual assessments
for a “leaky valve.” The patient denied any medication use.
On examination, Snellen visual acuity was OD 6/9 and OS
6/6. Pupils, confrontational visual fields, and cranial nerve
examination were normal. Posterior segment examination OD
revealed a large oblong, dense region of retinal pallor extending
from the temporal disc margin to the parafoveal region with
associated mild disc elevation (Fig. 1A). Posterior segment
examination OS showed 2 mid-peripheral white-centered hem-
orrhages within the supratemporal quadrant (Fig. 1B). Vitals
were measured: temperature: 37.1; pulses: right 110, left 111;
blood pressure: right 156/61, left 179/77. While taking periph-
eral pulses, a cyanotic left hand was noted with faint radial and
ulnar pulses (Fig. 1C). 10-2 Humphrey visual field revealed a
dense central inferior altitudinal scotoma respecting midline
(Fig. 2A). Optical coherence tomography of the macula dem-
onstrated a region of hyperreflectivity confined to the inner
layers of the retina (Fig. 2B). Urgent blood work revealed eryth-
rocyte sedimentation rate 59, C-reactive protein 60, white
blood cells 13, platelets 266. The ischemic cecocentral region
along with elevated inflammatory markers was concerning for
an inflammatory process.
Considering the unique presentation, additional probing into
the patient’s past medical history revealed a diagnosis of TA at
e288 CAN J OPHTHALMOL—VOL. 54, NO. 6, DECEMBER 2019
Can J Ophthalmol 2019;54:e285–e288
0008-4182/17/$-see front matter © 2019 Canadian Ophthalmological
Society. Published by Elsevier Inc. All rights reserved.
https://doi.org/10.1016/j.jcjo.2019.04.002
age 27, where she was started on high-dose prednisone but self-
weaned her prescribed treatment with the assistance of naturo-
pathic remedies. She cited poor doctor patient relationships as
the primary cause of treatment failure. She has remained off
treatment for 30 years without major disease flares.
After consultation with rheumatology, the patient was
started on oral prednisone 1 mg/kg and instructed to follow
up in 12 hours. On follow-up, the patient remained symp-
tomatic with a generalized anxious feeling and mild diaphore-
sis. Physical examination was repeated and revealed a palpable
subclavian thrill. She was admitted immediately to hospital
for diagnostic assessment.
An urgent computed tomography with contrast was per-
formed of the head and thorax. Focal stenosis of the right
subclavian artery (Fig. 3A) was present along with tortuos-
ity of the internal carotid artery and aortic wall thickening.
Angiographic imaging of upper limb revealed an occlusion
of the distal left brachial artery beyond the proximal fore-
arm (Fig. 3B). The patient was taken for urgent surgical
exploration where a thrombosed brachial artery was pres-
ent with posterior wall thinning and gross purulent mate-
rial. A bypass procedure was performed. Blood cultures
were taken, and the patient was started on intravenous
antibiotics. Histopathological assessment revealed a trans-
mural inflammation with wall rupture (Fig. 4A) and scat-
tered gram-positive cocci consistent with an infectious
aneurysm (Fig. 4B). Drawn blood cultures were positive
for streptococcus mitis. Transthoracic and transesophageal
echography failed to identify any evidence of endocarditis
or vegetative growths but demonstrated severe aortic valve
insufficiency secondary to aortic root dilatation.
During admission, a follow-up conversation with the
patient revealed a 1-month history of a recent febrile illness
after a minor dental procedure. This represents the likely
source of infection given streptococcus mitis is an oral
microbe. In light of the diagnostic and surgical findings, the
ocular manifestations were likely in keeping with a combined
ischemic and infectious process: untreated vasculitis resulting
in a nidus for bacterial overgrowth disseminated septic
emboli after an oral surgical procedure. Her medical course
improved in-hospital and at 2 months follow-up, both vision
and the central visual field defect improved (Fig. 5). She is
currently being managed by rheumatology, cardiology, and
ophthalmology.
TAGEDH1DISCUSSIONTAGEDEN
The case presented highlights the ischemic complications
of active TA with concurrent acute bacteremia. White-