Lupus profundus: not a benign disease

Abstract

Lupus profundus is an unusual clinical variant of cutaneous lupus erythematosus that has previously been described as a benign disease that follows a mild course. This report describes the extent of disease and associated comorbidities in patients with severe lupus profundus and systemic lupus erythematosus. Four cases of lupus profundus are reviewed and their associated systemic disease complications are highlighted. All four patients fulfylled at least four of the 11 criteria for systemic lupus erythematosus. One patient suffered from severe facial disfygurement and Parry-Romberg syndrome. Two patients developed nonhealing ulcers on the scalp. All four patients had scarring alopecia as well as depressed areas over large areas of their body surfaces. All patients were resistant to conservative therapy, and required long-term aggressive therapy. Clinical depression secondary to disfygurement was a major problem in three patients. Extensive lupus profundus may be associated with more serious systemic disease and warrants aggressive treatment early on to prevent permanent disfygurement and its resultant psychological consequences.