Childhood bullous systemic lupus erythematosus: unusual prognosis

Abstract

Background Bullous systemic lupus erythematosus (BSLE) has rarely been described in pediatric lupus population, and the real prevalence of childhood-onset BSLE has not been reported. BSLE is a rare manifestation of systemic lupus erythematosus and is very rare in children. BSLE affects women more often than men, reflecting the female preponderance in systemic lupus erythematosus. It most often manifests in the second through fourth decades of life, but it has also been reported in children and older adults. Patients and methods Herein, we describe a case of a 10-year-old female patient who presented with a 1-year history of recurrent itchy urticated skin eruption, which was followed 2 months later (after the first year) by a generalized blistering eruption with photosensitivity. Results Histopathologic examination revealed a subepidermal blister and papillary-tip neutrophil microabscesses. Immunofluorescence studies showed linear immunoglobulin G (IgG) and IgC deposition along the BMZ with weaker staining of IgA and IgM. Laboratory tests revealed positive ANA, dsDNA, anti-SM antibodies, elevated erythrocyte sedimentation rate, and proteinuria. Conclusion Although the course of BSLE is often remitting, but not in our case, treatment with dapsone is successful in most cases of BSLE.