Sclerodermiform Lupus Erythematosus: A Case Report of an Overlap Syndrome of Lupus Profundus and Morphea

Abstract

Sclerodermiform lupus erythematosus is a rare type 3 overlap syndrome between chronic cutaneous lupus erythematosus (CCLE) and morphea. The diagnostic criteria are shared histopathologic features of both CCLE and morphea. Here we reported a 49-year-old female presented with an indurated mass with intermittent pain on the left inner thigh for 3 years. Dermatological examination showed ill-defined hyperpigmented, indurated plaque with bound-down skin on the inner side of the left thigh. Skin biopsy revealed subcutaneous lobular lymphocytic infiltrate with lipomembranous and hyalinized fat necrosis mixed with thickened collagen. Alcian blue pH2.5 revealed an increase in dermal and subcutaneous mucin. Immunohistochemistry for CD123 was positive plasmacytoid dendritic cells in clusters. Serological studies showed positive antinuclear and anti-ds DNA antibodies. A diagnosis of sclerodermiform lupus erythematosus was made. Administration of prednisolone, methotrexate, and chloroquine resulted in gradually improved in 5 months. Previous reports of sclerodermiform lupus and morpheaform lupus were summarized.