Discoid lupus erythematosus of the periorbita: clinical dilemmas, diagnostic delays

Abstract

Purpose: Untreated periocular discoid lupus erythematosus (DLE), though very rare, may lead to significant morbidity with lid deformities, trichiasis, and symblepharon formation. We present the largest reported cohort of patients with biopsy-proven DLE solely affecting the periorbital region.

Methods: Observational case series of patients managed over a 7-year period (2004-10).

Results: Seven patients (one male) presented to the Adnexal Service at Moorfields Eye Hospital at a median age of 47 years (range 23-71 years); median interval from symptom onset to biopsy-proven diagnosis was 38 months (range 6-86 months). Changes in peripheral skin were present in 1 patient (occurring after the initial eyelid presentation) and the presenting periocular features were dissimilar across the group, these included: chronic blepharo-conjunctivitis, madarosis, atypical chalazia, depigmentation of the eyelid margin, or marked, persistent periocular oedema with dacryoadenitis.Two cases settled spontaneously, but five required systemic hydroxychloroquine or intralesional corticosteroid injections.

Conclusion: Periorbital DLE is rare and very varied in its presentation, the protean manifestations often resulting in significant diagnostic delay. All patients with unusual periocular skin disease and those with a refractory inflammatory dermopathy, should undergo biopsy of involved tissue(s), thus leading to earlier diagnosis and prevention of permanent cicatricial periocular changes.

Figure 1

(Patient1) (a) Violaceous swelling with eczematous changes at presentation; (b) Coronal orbital CT images demonstrate right lacrimal gland, superior and lateral recti enlargement. (c) Facial swelling almost completely resolved without scarring; (Patient 2) (d) Localised lower lid margin depigmentation, preserved lashes, no erythema; (Patient 3) (e) Diffusely bulky left upper eyelid with areas of depigmentation and atrophy; lateral lid margin destruction and madarosis. (Patient 4) (f) Lower lid at presentation shows erythematous, scaly, discoid lesion with madarosis.