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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.
      • Pearson M.
      • Selby J.
      • Katz K.
      • et al.
      Clinical, epidemiologic, histopathologic and molecular features of an unexplained dermopathy.
      Patients often have accompanying musculoskeletal and neurological manifestations, suggested by some authors to resemble the symptoms of Lyme disease.
      • Middelveen M.J.
      • Fesler M.C.
      • Stricker R.B.
      History of Morgellons disease: from delusion to definition.
      • Middelveen M.
      • Burugu D.
      • Poruri A.
      • et al.
      Association of spirochetal infection with Morgellons disease.
      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.
      • Middelveen M.J.
      • Fesler M.C.
      • Stricker R.B.
      History of Morgellons disease: from delusion to definition.
      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.
      • Pearson M.
      • Selby J.
      • Katz K.
      • et al.
      Clinical, epidemiologic, histopathologic and molecular features of an unexplained dermopathy.
      Their proposed definition is: reported fibers or other solid materials protruding from the skin, and skin lesions or disturbing skin symptoms.
      • Pearson M.
      • Selby J.
      • Katz K.
      • et al.
      Clinical, epidemiologic, histopathologic and molecular features of an unexplained dermopathy.
      To the best of our knowledge, there is only one previous case of Morgellons disease reported in the ophthalmic plastic literature.
      • Sandhu R.
      • Steele E.
      Morgellons disease presenting as an eyelid lesion.
      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 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
      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.
      • Huang Y.
      • Wang C.
      • Potenziani S.
      • Hsu S.
      Lichen planus of the eyelids: a case report and review of the literature.
      Furthermore, lichen planus favours the flexor surfaces, trunk, and oral or genital mucosa with only 6 reported cases isolated to the eyelids.
      • Huang Y.
      • Wang C.
      • Potenziani S.
      • Hsu S.
      Lichen planus of the eyelids: a case report and review of the literature.
      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)
      American Psychiatric Association
      Diagnostic and Statistical Manual of Mental Disorders.
      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.
      • Sandhu R.
      • Steele E.
      Morgellons disease presenting as an eyelid lesion.
      • de Menezes Bedran E.
      • Correia Pereira M.
      • Bernardes T.
      Ectropion.
      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.
      • Pearson M.
      • Selby J.
      • Katz K.
      • et al.
      Clinical, epidemiologic, histopathologic and molecular features of an unexplained dermopathy.
      • Robles D.
      • Olson J.
      • Combs H.
      • et al.
      Morgellons disease and delusions of parasitosis.
      • Foster A.A.
      • Hylwa S.A.
      • Bury J.E.
      • et al.
      Delusional infestation: clinical presentation in 147 patients seen at Mayo Clinic.
      Laboratory tests indicative of inflammation or infection are routinely normal and skin biopsies reveal nonspecific findings.
      • Pearson M.
      • Selby J.
      • Katz K.
      • et al.
      Clinical, epidemiologic, histopathologic and molecular features of an unexplained dermopathy.
      • Robles D.
      • Olson J.
      • Combs H.
      • et al.
      Morgellons disease and delusions of parasitosis.
      Furthermore, the CDC could not identify any underlying medical or infectious causes and found that fibers were consistent with superficial skin or cotton.
      • Pearson M.
      • Selby J.
      • Katz K.
      • et al.
      Clinical, epidemiologic, histopathologic and molecular features of an unexplained dermopathy.
      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.
      • Middelveen M.J.
      • Fesler M.C.
      • Stricker R.B.
      History of Morgellons disease: from delusion to definition.
      • Middelveen M.
      • Burugu D.
      • Poruri A.
      • et al.
      Association of spirochetal infection with Morgellons disease.
      • Savely V.R.
      • Leitao M.M.
      • Stricker R.B.
      The mystery of Morgellons disease: infection or delusion?.
      Our observations for this case are consistent with a delusional etiology.
      The medical management of Morgellons involves low-dose typical and atypical antipsychotics.
      • Driscoll M.
      • Rothe M.
      • Grant-Kels J.
      • Hale M.
      Delusional parasitosis: a dermatologic, psychiatric, and pharmacologic approach.
      • Koo J.
      • Lee C.
      Delusions of parasitosis: a dermatologist's guide to diagnosis and treatment.
      • Lepping P.
      • Russell I.
      • Freudenmann R.
      Antipsychotic treatment of primary delusional parasitosis.
      Although antipsychotics are considered first-line treatment, a systematic review found limited evidence for their effectiveness.
      • Lepping P.
      • Russell I.
      • Freudenmann R.
      Antipsychotic treatment of primary delusional parasitosis.
      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.
      Government of Alberta
      Tick Surveillance 2017 Summary.
      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.

      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].

      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,
      • Sandhu R.
      • Steele E.
      Morgellons disease presenting as an eyelid lesion.
      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.
      • Sandhu R.
      • Steele E.
      Morgellons disease presenting as an eyelid lesion.
      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.
      • Driscoll M.
      • Rothe M.
      • Grant-Kels J.
      • Hale M.
      Delusional parasitosis: a dermatologic, psychiatric, and pharmacologic approach.
      To obtain patient consent and compliance with psychiatric treatment, many authors advocate establishing a strong therapeutic alliance.
      • Robles D.
      • Olson J.
      • Combs H.
      • et al.
      Morgellons disease and delusions of parasitosis.
      • Driscoll M.
      • Rothe M.
      • Grant-Kels J.
      • Hale M.
      Delusional parasitosis: a dermatologic, psychiatric, and pharmacologic approach.
      • Koo J.
      • Lee C.
      Delusions of parasitosis: a dermatologist's guide to diagnosis and treatment.
      Conducting a thorough and complete skin and ophthalmological assessment validates patient concerns, and rules out other organic causes of cicatricial lid changes.
      • Driscoll M.
      • Rothe M.
      • Grant-Kels J.
      • Hale M.
      Delusional parasitosis: a dermatologic, psychiatric, and pharmacologic approach.
      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.
      • Robles D.
      • Olson J.
      • Combs H.
      • et al.
      Morgellons disease and delusions of parasitosis.
      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.

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