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Unilateral Eyelid Discoid Lupus Erythematosus

Ricotti, Carlos MD; Tozman, Elaine MD; Fernandez, Anthony MD, PhD; Nousari, Carlos H MD

The American Journal of Dermatopathology: October 2008 - Volume 30 - Issue 5 - p 512-513
doi: 10.1097/DAD.0b013e318181593c
Letters to the Editor

University of Miami L. Miller School of Medicine Department of Dermatology Miami FL

Dermpath Diagnostics, South Florida, FL

To the Editor:

Discoid lupus erythematosus (DLE) involving palpebral areas is estimated to occur in up to 5% of all patients with mucocutaneous DLE.1 These patients typically present with bilateral eyelid involvement and other associated mucocutaneous DLE lesions. Solely unilateral eyelid DLE is exceedingly rare and more commonly associated with delayed diagnosis and potential increased morbidity. We present a patient with unilateral eyelid DLE associated with madarosis, scarring, and mucosal involvement.

A 38-year-old, otherwise healthy, woman presented with a 5-year history of an enlarging left lower eyelid lesion associated with pain, edema, and epiphora (Fig. 1). She denied other mucocutaneous lesions, loss of visual acuity, or constitutional symptoms. Previous therapies had resulted in minimal response and included topical triamcinolone 0.1% ointment, mupirocin ointment, and tobramycin plus dexamethasone eyedrop solutions. Physical examination revealed an indurated, erythematous, scaly plaque of the outer aspect of the left lower eyelid. Significant madarosis, early entropion, contiguous erythema, and edema of the inferior palpebral conjunctiva were also noted. No symblepharon or other ocular abnormality was detected on slit lamp and funduscopic examination. Ancillary studies including complete blood cell count, C3, C4, CH50, sedimentation rate, creatinine, urine analysis, antinuclear antibody, anti-dsDNA, rheumatoid factor, anti-ribonucleoprotein, anti-Sm, anti-Ro, and anti-La were negative or within normal limits. Histological evaluation of a 3-mm lesional punch biopsy specimen revealed an interface dermatitis associated with follicular involvement, superficial and deep perivascular lymphocytic infiltrate, increased dermal mucin confirmed with colloidal iron stain, and significant thickening of the epidermal and adnexal basement membrane confirmed with periodic acid-Schiff stain. Direct immunofluorescence evaluation of a 3-mm punch biopsy obtained from lesional skin revealed dense granular immunoglobulin M, C3, weaker immunoglobulin G, and C5b-9 deposits along the epidermal and adnexal basement membrane zone, confirming the diagnosis of DLE (Figs. 2A, B).

FIGURE 1

FIGURE 1

FIGURE 2

FIGURE 2

She was placed on 40-mg prednisone with a tapering-off regimen for more than 4 months. Mycophenolate mofetil 1 g twice daily and hydroxychloroquine 200 mg twice daily were concomitantly initiated as a corticosteroid-sparing therapy. On 5-month follow-up, significant improvement was noted while she continues on corticosteroid-sparing adjuvants.

Eyelid involvement by DLE lesions has significant morbidity including madarosis, ectropion, entropion, scarring, cicatrizing conjunctivitis with symblepharon formation, and eventually blindness.2 Furthermore, DLE lesions that present solely on the eyelids frequently lack the typical clinical morphologic findings of this form of chronic cutaneous lupus erythematosus, imposing a diagnostic challenge and potential delayed therapy.3 Thus, awareness of this atypical presentation of DLE along with adequate sampling including the deep dermis and subcutis for both routine histology and direct immunofluorescence evaluation is crucial for accurate clinical pathologic correlation.

Carlos Ricotti, MD

University of Miami L. Miller School of Medicine Department of Dermatology Miami FL

Elaine Tozman, MD

University of Miami L. Miller School of Medicine Division of Rheumatology Miami FL

Anthony Fernandez, MD, PhD

University of Miami L. Miller School of Medicine Department of Dermatology Miami FL

Carlos H. Nousari, MD

Dermpath Diagnostics South Florida Pompano Beach FL // University of Miami L. Miller School of Medicine Department of Dermatology Miami FL

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REFERENCES

1. Burge SM, Frith PA, Juniper RP, et al. Mucosal involvement in systemic and chronic cutaneous lupus erythematosus. Br J Dermatol. 1989;121:727-741.
2. Acharya N, Pineda R, Uy HS, et al. Discoid lupus erythematosus masquerading as chronic blepharoconjunctivitis. Ophthalmology. 2005;112:e19-e23.
3. Tosti A, Tosti G, Giovannini A. Discoid lupus erythematosus solely involving the eyelids: report of three cases. J Am Acad Dermatol. 1987;16:1259-1260.
© 2008 Lippincott Williams & Wilkins, Inc.