Unilateral eyelid discoid lupus erythematosus.

To the Editor: Discoid lupus erythematosus (DLE) involving palpebral areas is estimated to occur in up to 5% of all patients with mucocutaneous DLE. 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, antiribonucleoprotein, 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). 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 corticosteroidsparing 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. Furthermore, DLE lesions that present solely on the eyelids FIGURE 1. Involving the outer aspect of left lower eyelid, physical examination showed an indurated, pink, erythematous, scaly plaque. Significant madarosis, early entropion, contiguous erythema, and edema of the inferior palpebral conjunctiva were also noted. FIGURE 2. A, Routine histological examination of lesional skin punch biopsy showed a interface dermatitis with hyperkeratosis, few dyskeratotic keratinocytes, thickened epidermal and adnexal basement membrane zones, increased superficial and deep mucin, and perifollicular sebaceous lymphocytic infiltrate. B, On direct immunofluorescence examination of lesional skin punch biopsy, immunoglobulin M, immunoglobulin G, and C3 heavy and thick granular deposition along the epidermal and adnexal basement membrane zone were noted. Few cytoid elements were noted. Indirect immunofluorescence showed no circulating antiepithelial antibodies.