Lupus erythematosus (LE) is an autoimmune disease that has a constellation of clinical manifestations from cutaneous to multi-organ systemic involvement. Childhood-onset SLE is an uncommon, lifelong autoimmune disease that is difficult to diagnose due to its multisystem involvement and multiplicity of clinical manifestations. In children, adolescent girls are most commonly affected with a peak incidence at about 12 years of age. Bullous Systemic Lupus Erythematosus (BSLE) is a rare manifestation of SLE with few cases described in childhood.

We report a case of a 6-year-old girl with an unrecalled history of chronic livedo re:cularis and a 3-month history of non-scarring alopecia who developed multiple minimally pruritic vesicles on the face and neck. The lesions increased in number, size, and distribution, becoming excoriated. The Nikolsky and Asboe-Hansen signs were negative. There was noted oral mucosal involvement and bilateral knee pain at rest with no joint swelling. A 3-mm skin punch biopsy of a vesicle from the upper back revealed an intraepidermal blister. Direct immunofluorescence of perilesional skin showed linear deposits of IgG (+2), C3 (+2), granular IgM (+2), Linear IgA and granular fibrinogen (+2) at the basement membrane zone, consistent with bullous systemic lupus erythematosus. ANA was positive at 1:160 (speckled pattern). We started the patient on dapsone 1.5 mg/kg/day. The patient was co-managed with Pediatric Rheumatology and oral prednisone at 2 mg/kg/day, hydroxychloroquine 50 mg once a day, mycophenolate mofetil 750 mg once a day and enalapril 5 mg once a day were prescribed. The lesions became dry and flat and healed without scarring with no new eruptions noted.

BSLE is uncommon in younger individuals. They usually present with a widespread symmetrical distribution of vesicular skin lesions and frequently favor the sun-exposed areas. We present here a rare case of BSLE in a young individual with an unusual histologic presentation. Treatment with dapsone, corticosteroids, hydroxychloroquine and mycophenolate mofetil afforded complete resolution in our case.