BACKGROUND
Bullous pemphigoid is an autoimmune blistering disease, with relapses, isolated or associated with other
autoimmune diseases such as
systemic lupus erythematosus (SLE). Joint manifestations rapidly respond to small or moderate doses of
corticosteroids, whereas
skin manifestations usually respond to
antimalarial drugs. CASE REPORT We describe the clinical case of an 11-year-old girl with SLE. She showed bullous skin lesions with
arthralgia, mild
proteinuria, resolved after
steroid treatment. At the tapering of her
prednisone dose, the patient had new skin lesions requiring an increased dose of
prednisone. She started
dapsone at the dosage of 1 mg/kg/day, maintaining low dose
prednisone; this treatment was successfully followed by the dramatic disappearance of skin lesions and limb
pain. CONCLUSIONS Bullous skin lesions can represent the first clinical presentation of pediatric SLE and could influence the treatment and the outcome of these patients. This case showed an atypical course as both
skin manifestations and
arthritis promptly and persistently resolved with
dapsone without the use of high-dose
glucocorticoids. Only a few cases of patients with SLE associated with
bullous pemphigoid have been reported in the literature, and very few in the pediatric population.