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Morgellons disease is a somewhat controversial and poorly understood skin disorder characterized by crawling sensations associated with slow-healing, ulcerated, filamentous skin lesions.
Although several 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.
The Centers for Disease Control and Prevention (CDC) suggested that “Morgellons” is a lay term used to describe an unexplained constellation of symptoms, with manifestations primarily involving the skin.
We present a case of Morgellons disease with significant cicatricial changes resulting in exposure keratopathy, corneal perforation, and endophthalmitis leading to enucleation.
A 63-year-old Caucasian woman presented to the emergency on-call clinic with a large descemetocele, diffuse corneal 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 occipitoparietal infection “creeping” to her face.
She was admitted to hospital for fortified topical antibiotics; 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 consistent with pyogenic endophthalmitis (Fig. 1).
Fig. 1Histopathological 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.
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 tobramycin/dexamethasone BID were started; unfortunately, her implant exposure did not heal, and 1 month later, she underwent 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 ectropion (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 eyelid, 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).
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.
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 characteristic changes suggestive of lichen planus were absent, including hypergranulosis, hyperkeratosis, and lymphohistiocytic infiltrate at the dermal-epidermal junction.
After multiple biopsies and unsuccessful fibre visualization, infectious disease was consulted to rule out underlying infectious etiologies, all of which rendered 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, exposure 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 ulcerated skin and the authors were unable to visualize any fibers. Subsequently, a medial third permanent tarsorrhaphy was performed as she declined eyelid retraction repair. Repeated skin trauma resulted in stretching and distortion of the tarsorrhaphy, causing tenting of the eyelid medially, and she also developed an 8 mm by 8 mm area over the right lateral brow with bone exposure. Her corneal surface continued to decompensate and she developed a 1.6 mm by 2.4 mm left corneal ulcer. She was admitted to hospital and started on fortified topical cefazolin and tobramycin in conjunction with systemic doxycycline aimed at inhibiting matrix metalloproteinases to limit further collagenolysis and corneal thinning. After an unsuccessful attempt to extend the tarsorrhaphy to the middle 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)
and agreed with the diagnosis of Morgellons disease. Risperidone and quetiapine were trialled on 2 separate occasions, but discontinued 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.
After ruling out the above with a proper evaluation, biopsy, culture, and subspecialty input, it is important to consider Morgellons disease in a patient presenting with cicatricial eyelid changes.
The etiology of Morgellons disease is currently unknown, although many dermatologists and psychiatrists consider it a subtype of delusional parasitosis.
Furthermore, the CDC could not identify any underlying medical or infectious causes and found that fibers were consistent with superficial skin or cotton.
In contrast, some authors suggest that Borrelia 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 antibiotic therapy.
Given the different proposed disease processes, treatment to cover an infectious etiology may be considered. In our opinion, antimicrobial 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.
Additionally, only 87 cases of Lyme disease were reported in Alberta from 1991 to 2016, all of which were acquired while travelling outside of Alberta.
or, in this case, implant exposure after enucleation. Ectropion repair was deferred in the previously published case of Morgellons in consideration of wound dehiscence and an aggressive eye rubbing history.
For Morgellons patients with cicatricial lid changes, it may be best to defer surgery and opt for medical management with antibiotic ointment and good lubrication if exposure keratopathy is present. A referral to psychiatry is also key.
Conducting a thorough and complete skin and ophthalmological assessment validates patient concerns, and rules out other organic causes of cicatricial lid changes.
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.
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 materials discussed in this article.
References
Pearson M.
Selby J.
Katz K.
et al.
Clinical, epidemiologic, histopathologic and molecular features of an unexplained dermopathy.
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