Sclerodermiform linear lupus erythematosus: A distinct entity or coexistence of two autoimmune diseases?

The coexistence of features of chronic cutaneous lupus erythematosus and scleroderma in the same skin lesions is very infrequent and has only been reported in 3 patients. This exceedingly rare condition has been named "sclerodermiform linear lupus erythematosus." We describe a new case of this dermatosis. Although the cause remains obscure, possible explanations include mosaicism following Blaschko's lines or the transfer of microchimerisms that mount a chronic graft-versus-host-like reaction. We suggest that these features may be a distinct clinicopathologic disorder characterized by an initial lichenoid reaction followed by the production of excessive, abnormal connective tissue.