Wendy chen md phd pgy 3 charleen t chu md phd neuropathology
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Wendy Chen, MD, PhD: PGY-3 Charleen T. Chu, MD, PhD: Neuropathology. Ocular Pathology Clinical Case January 2010. History of Present Illness. 50 yo Caucasian male presenting with right lower eyelid erythema and thickening. Itchy Erythema fluctuates, worse after sun exposure

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Wendy chen md phd pgy 3 charleen t chu md phd neuropathology

Wendy Chen, MD, PhD: PGY-3

Charleen T. Chu, MD, PhD: Neuropathology

Ocular PathologyClinical CaseJanuary 2010


History of present illness
History of Present Illness

  • 50 yo Caucasian male presenting with right lower eyelid erythema and thickening.

    • Itchy

    • Erythema fluctuates, worse after sun exposure

    • No change in vision

    • Some AM eyelid matting/crusting


Hpi cont d
HPI cont’d

  • Seen by dermatologist in 2006 -- bilateral periorbital rash, erythematous and itchy, scaling plaques, worsened by sun exposure.

  • ? Rosacea ---> treated with Akne-mycin (erythromycin 2%) without improvement.

  • Punch biopsy of LEFT lower eyelid


Hpi cont d1
HPI cont’d

  • Dermatopathology report:

    “lichenoid dermatitis with deep perivascular extension of the infiltrate . . . lupus erythematosis vs. lichenoid photodermatitis”

  • Subsequently,

    • PAS stain: thickened basement membrane

    • Colloidal iron: focal increase in dermal mucin

    • Favoring discoid lupus erythematosis


Hpi cont d2
HPI cont’d

  • Treated with Protopic (tacrolimus 0.1%) – bilateral periorbital rash improved.

  • Serologic testing:

    • ANA, RF, Anti-DNA, Anti-histone, Anti-SS A/B negative.

    • C3, C4 WNL

    • CBC, LFTs, BMP WNL

    • RPR non-reactive


Hpi cont d3
HPI cont’d

  • Over the next 3 years, pt continued to have intermittent flares of periorbital, facial, and scalp rashes ---> continued Protopic.

  • In 2009, presented to dermatology again with persistent RLL erythema and thickening accompanied by erythematous pustules of the face ---> acne rosacea

  • To ophthalmology for second opinion


Medical history
Medical History

  • Past Medical/Surgical History

  • Acne rosacea

  • Lupus erythematosis, DISCOID, not systemic

  • Anxiety, Depression

  • S/P hernia repair

  • Past Ocular History

  • S/P left periorbital skin bx

  • Hyperopia

  • Astigmatism

  • Social History

  • Tob – none

  • EtOH – social use

  • Drugs – none

  • Excessive sun exposure and multiple severe sunburns prior to age 18

  • Medications

  • Topical tacrolimus 0.1% prn

  • Lexapro

  • Ativan


Initial exam
Initial Exam

Va CC: 20/20 OU

Pupils: No APD

IOP: 14 OU

EOM: Full OU

CVF: Full OU



Differential diagnosis
Differential Diagnosis

  • Ocular rosacea – typically bilateral, but can be asymmetric.

  • Discoid lupus without systemic involvement.

  • Infectious – viral, bacterial, fungal.

  • Malignancy – basal cell carcinoma, squamous cell carcinoma, sebaceous adenocarcinoma.


Approach to management
Approach to management

  • Rosacea-associated blepharitis ---> Blephamide BID with resolution of symptoms.

  • Returned 5 months later with RLL recurrence (slow return of symptoms).

  • Referred to oculoplastics for biopsy.


Work up
Work-up

  • Wedge resection of lateral portion of the RLL lesion in Oct 2009.

  • Conjunctival cultures taken:

    • Fungus culture negative

    • Virus culture negative

    • Bacterial culture with light coag neg Staph

    • Adenovirus PCR negative

    • HSV1/2 PCR negative


Hyperkeratosis

Acanthosis

Squamous cell nests and strands infiltrating the dermis






Pathology report
Pathology report

  • EYELID RIGHT LOWER, WEDGE BIOPSY

    • A. SUPERFICIALLY INVASIVE SQUAMOUS CELL CARCINOMA (0.2 CM, 0.1 CMTHICK), WELL DIFFERENTIATED

    • NO ANGIOLYMPHATIC OR PERINEURAL INVASION PRESENT.

    • MARGINS FREE OF TUMOR.


Clinical course cont d
Clinical course cont’d

  • Pt continued to have persistent RLL erythema and thickening.

  • Re-excision of adjacent area performed Nov. 2009, given prior diagnosis ---> suture with foreign body giant cell reaction, acute and chronic inflammation and fibrosis.


Clinical course cont d1
Clinical course cont’d

  • Started po Doxycycline 100mg daily for further treatment of ocular rosacea.

  • Patient was followed q4 months for 1 yr with waxing and waning progression of RLL lesion.

  • Most recent visit revealed a change in appearance of RLL.


Prior biopsy site

Vertical extension of the lesion with minor distortion of lid architecture


Management cont d
Management cont’d

  • Given the recent change in the appearance of the lesion and prior diagnosis of carcinoma, a repeat wedge resection was performed in January 2011 on the medial portion of the RLL lesion.



Maturation & narrow strand-like extensions suggest pseudocarcinomatous hyperplasia

Thick basement membrane


Re review of prior excisions
Re-review of prior excisions pseudocarcinomatous hyperplasia

  • Review of initial wedge excision revealed that the diagnosis of carcinoma may have been incorrect.

    • Prolonged clinical history of rashes, chronic inflammation and suspicion of DLE were not known to the original pathologist.

  • Both excisions showed:

    • SQUAMOPROLIFERATIVE LESIONS WITH PSEUDOEPITHELIOMATOUS HYPERPLASIA.


Squamous cell carcinoma
Squamous pseudocarcinomatous hyperplasia Cell Carcinoma

  • 10-40X less common than basal cell carcinoma.

  • Typically arise from actinic keratoses.

  • Lower eyelid most common ocular site.

  • Histologic characteristics:

    • Hyperkeratosis, acanthosis

    • Interface dermatitis, infiltrative nests and strands

    • Keratinocyte nuclear hyperchromasia and maturational ayptia, mitotic figures

    • Keratin pearls, dyskeratotic cells


Pseudocarcinomatous hyperplasia also known as pseudoepitheliomatous hyperplasia
Pseudocarcinomatous pseudocarcinomatous hyperplasia Hyperplasia (also known as pseudoepitheliomatous hyperplasia)

  • Chronic inflammation can result in histologic changes that mimic invasive squamous carcinoma.

  • Features that can help differentiate PCH/PEH from SCC:

    • Narrow, strand-like infiltration of epithelium

      • Tangential section can result in isolation from surface

    • Lack of dysplastic hyperchromatic nuclei, lack of maturational atypia

      • Reactive atypia – pale nuclei with uniform nucleoli


Eyelid involvement by chronic cutaneous lupus ccl
Eyelid involvement by chronic pseudocarcinomatous hyperplasiacutaneous lupus (CCL)

  • Systemic lupus more commonly causes corneal lesions, retinal vasculopathy

    • keratoconjunctivitis sicca, peripheral ulcerative keratitis, interstitial keratitis

  • Very rarely can give isolated lid lesions that mimic malignancy

  • CCL variants

    • Discoid lupus erythematosis – plaque lesions

    • Lupus erythematosis profundus (panniculitis)

    • Systemic disease - idiopathic orbital edema


Eyelid involvement by chronic cutaneous lupus
Eyelid involvement by chronic pseudocarcinomatous hyperplasiacutaneous lupus

  • Unlike regular pseudoepitheliomatous hyperplasia, significant cytologic atypia (N/C ratio, hyperchromasia, mitoses) can occur.

  • Features that help differentiate DLE from SCC:

    • History of chronicity, rashes

      • SCC shows rapid onset/growth (< 6 mo), nodular or ulcerative changes.

    • Intradermal mucin (colloidal iron)

    • Thickened basement membrane (PAS)

      Papalas et al. “Cutaneous Lupus Erythematosus of the Eyelid as a Mimic of Squamous Epithelial Malignancies” Ophthal Plast Reconstr Surg 2010.


Distinguishing features cont d
Distinguishing features cont’d pseudocarcinomatous hyperplasia

  • Features that help differentiate CCL from SCC:

    • Perifollicular and acrosyringeal inflammation

    • Follicular plugging

    • Vacuolar interface change

    • Compact orthokeratosis

  • The “helpful” features may not be present or may not be recognized without a high index of suspicion

    • 37% of cutaneous LE cases incorrectly interpreted initially even by Board certified dermatopathologists

      Zedek et al. “Cutaneous Lupus Erythematosis simulating squamous neoplasia: The clinicopathologic conundrum and histopathologic pitfalls” J Am Acad Dermatol 2007; 56: 1013-20.


Summary
Summary pseudocarcinomatous hyperplasia

  • Persistent unilateral lower lid erythema/thickening despite treatment of discoid lupus and ocular rosacea warrants biopsy.

    • SCC can develop in lesions of discoid LE

  • The histologic DDx includes actinic keratosis/SCC, lichen planus-like keratosis & PCH/PEH

    • Combination of cytologic atypia from lupus and PCH/PEH (which normally lacks atypia) is a diagnostic pitfall

    • Chronic history and suspicion of lupus would raise the awareness threshold to prevent overcalling the lesion

  • Providing clinical history is key to avoiding misdiagnosis >> rare mimics of a common neoplasm


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