Lupus profundus is an unusual clinical variant of cutaneous lupus erythemat
osus that has previously been described as a benign disease that follows a
mild course. This report describes the extent of disease and associated com
orbidities in patients with severe lupus profundus and systemic lupus eryth
ematosus. Four cases of lupus profundus are reviewed and their associated s
ystemic disease complications are highlighted. All four patients fulfilled
at least four of the 11 criteria for systemic lupus erythematosus. One pati
ent suffered from severe facial disfigurement and Parry-Romberg syndrome. T
wo patients developed nonhealing ulcers on the scalp. All four patients had
scarring alopecia as well as depressed areas over large areas of their bod
y surfaces. All patients were resistant to conservative therapy, and requir
ed long-term aggressive therapy. Clinical depression secondary to disfigure
ment was a major problem in three patients. Extensive lupus profundus may b
e associated with more serious systemic disease and warrants aggressive tre
atment early on to prevent permanent disfigurement and its resultant psycho
logical consequences.