A Woman with Hyperpigmented Plaques of the Face

History: A 56-year-old African American female presented for evaluation of a 10-year history of dark patches involving the forehead, malar cheeks, nose, ears, upper back, and extremities. She endorsed daily pruritus of the ears, face, and back that was accompanied by an intermittent burning sensation. Prior treatment with hydroxychloroquine, topical, intralesional and systemic corticosteroids was of little benefit. Exam: On the face, in a photo-distributed pattern, there were hyperpigmented patches and plaques with verrucous like texture. There were hyperpigmented macules of the bilateral conchal bowls, helix and scaphoid fossa. All proximal nail folds on the fingers exhibited a variable level of edema and erythema. The oral mucosa was uninvolved. Diagnostics:Labs revealed an ANA titer of 1:1280 (speckled pattern); Complement levels and activity, dsDNA, ENA, SSA/B, cardiolipin studies, and urinalysis were all normal. A punch biopsy of the right medial canthus was performed revealing few pigmented particles in the stratum corneum. There was a markedly thickened basement membrane with underlying dermal melanophages scattered throughout the papillary dermis. No evidence of active inflammation was observed. A Perls’ stain was negative for the presence of iron. Course and therapy:The patient had previously been diagnosed with discoid and systemic lupus erythematosus (SLE) ten years ago at an outside facility. While her SLE has been quiescent since 2010, her facial lesions have been recalcitrant to hydroxychloroquine and multiple corticosteroids. At the time of presentation, the differential diagnoses included: active DLE / SLE, hydroxychloroquine-induced hyperpigmentation, and lichen simplex chronicus secondary to rubbing. The recent biopsy did not show any evidence of an active inflammatory process or scarring; only remnants of likely previous lupus. She is currently improving on mycophenolate mofetil 1500 mg BID, hydroxychloroquine 200 mg BID alternating with 200 mg QD, tazarotene 0.1% gel nightly to hyperkeratotic areas of the face, and desonide 0.05% ointment BID PRN for pruritus. Discussion: Lesions of discoid lupus erythematosus (DLE) are one of the most common cutaneous manifestations of lupus usually involving the scalp, face, and ears and more rarely the mucosal surfaces. These lesions are characterized by coin-shaped, well-demarcated plaques that progress into dyspigmented scars. Studies estimate that about 10-20% of patients who present with discoid lesions progress to meet criteria for systemic lupus erythematosus (SLE), with the majority of these patients progressing within 5 years. Hypertrophic DLE, a rare variant occurring in 2% of patients with DLE, is characterized by hyperkeratotic, verrucous plaques predominately involving the extensor surfaces of the upper extremities, trunk, and face. Hypertrophic DLE is often more resistant to treatment.Topical corticosteroids and topical calcineurin inhibitors are first line treatment for DLE in addition to strict photoprotection. Intralesional corticosteroids may be of benefit in patients with chronic DLE lesions refractory to topical agents. There is also data to suggest that pulsed dye laser (PDL) with a wavelength of 585-595 nm and topical retinoids, such as tazarotene, may be effective. Antimalarials are typically used as first line systemic therapy; typically, hydroxychloroquine or less commonly, chloroquine, is given, either as monotherapy or as combination therapy with quinacrine. Retinal toxicity and hydroxychloroquine-induced pigmentation are potential side effects of these medications. Some data suggest that methotrexate and mycophenolate mofetil may be effective as second-line options. Acitretin, thalidomide, and isotretinoin have been reported to be efficacious in treating hypertrophic DLE. There have also been reports of improvement of severe and recalcitrant DLE with ustekinumab.https://scholarlycommons.henryford.com/merf2019caserpt/1010/thumbnail.jp