Track

Case Reports

Abstract

A 53-year-old woman with seropositive rheumatoid arthritis presented with worsening fatigue and a few years history of pruritic cutaneous eruptions favoring the upper extremities. Stable immunosuppressive therapy included methotrexate 20mg PO weekly, prednisone 5mg PO daily, and hydroxychloroquine 200mg PO twice daily. Past empiric topicals (clobetasol 0.05%, fluocinonide 0.05% ointments) effected partial cutaneous resolution. Cutaneous examination revealed bilateral arm indurated red-brown scaly papules and plaques with focal yellow hue. Punch biopsy demonstrated compact hyperkeratosis, lichenoid interface dermatitis, thickened basement membrane zone, superficial/deep perivascular periadnexal lymphohistioplasmacytic inflammation. Concurrent nodular and interstitial dermal aggregates of foamy macrophages were present. Colloidal iron demonstrated increased dermal mucin. Special staining (AFB/Gram/PAS/GMS) was negative for microbial forms. Clinicopathologic correlation supported discoid lupus erythematosus with xanthomatized macrophages. Peripheral bloodwork revealed leukopenia (3.0x10³/uL) and normocytic anemia (hemoglobin 10.1 g/dL). C-reactive protein and erythrocyte sedimentation rate were newly elevated (4.53 mg/dL and 130 mm/hr, respectively). Lipid panel was grossly normal. Historic labs five years prior demonstrated positive ANA (1:640, nucleolar pattern) with negative extractable nuclear antigens (anti-dsDNA/sm/RNP/SSA/SSB/Scl-70 antibodies). ANA positivity was previously attributed to etanercept, with last anti-TNF alpha inhibitor administration two years ago for rheumatoid arthritis. Workup for systemic lupus erythematosus (SLE) is ongoing. This case serves to expand upon rare reports of xanthomatized macrophages in the setting of chronic cutaneous lupus erythematosus, and may represent the presenting sign of SLE. Intracellular lipid release/uptake in the setting of chronic lichenoid inflammation is a proposed mechanism. Clinicopathologic correlation is key to diagnosing cutaneous lupus erythematosus with atypical features.