Seven-year itch: a perplexing case of lichen planus-lupus erythematosus overlap syndrome

Abstract

Lichen planus-lupus erythematosus overlap syndrome is a rare disorder characterized by clinical and histopathological features of both lichen planus (LP) and lupus erythematosus (LE). Cutaneous lesions commonly affect the distal arms, legs, face, and trunk and these plaques are often large, scaly, painful, and atrophic, often exhibiting hypopigmentation or a red to blue-violet color. We report a case of LP-LE overlap syndrome diagnosed in a man previously believed to have atypical lichen planus who presented with an exacerbation of exuberant pruritic erythematous scaly plaques. The patient had six separate skin biopsies all of which displayed features of LP. Because the clinical symptoms did not correlate to the histopathological picture, a seventh skin biopsy with direct immunofluorescence (DIF) was performed and immunologic markers measured. The DIF demonstrated early lupus bands; serologic testing exhibited elevated ANA and anti-SSA. These findings established the diagnosis of LP-LE overlap syndrome. The patient was started on hydroxychloroquine with short-term trials of oral prednisone during disease flares, which took place in the first three months of treatment.