Pediatric Lichen Planus Pemphigoides in a Child With Sickle Cell Disease

Lichen planus pemphigoides (LPP) is a rare autoimmune subepidermal blistering disorder that combines lichenoid lesions of lichen planus with immunopathologic features of pemphigoid disease. We report an 8-year-old boy with homozygous sickle cell disease and severe atopic dermatitis who developed diffuse pruritic violaceous papules followed by tense bullae; subsequent histopathologic, immunofluorescence, and serologic findings confirmed LPP. The patient was initially treated with cyclosporine, with marked clinical improvement within 2-3 weeks. Dupilumab was subsequently initiated during the cyclosporine taper, and no recurrent bullae were observed through approximately 8 months of follow-up.

Abstract from PubMed