Dystrophic Epidermolysis Bullosa Pruriginosa Presenting with Flagellate Scarring Pattern Before and After Treatment

Abstract

Introduction: Dystrophic Epidermolysis Bullosa Pruriginosa (DEB-Pr) is a rare subtype of DEB which is characterized by intense pruritus and leading to skin blistering and nodular or lichenoid lesions most prominent on the extremities, especially shins of tibia. Toenail dystrophy is also frequently observed, but not necessary for diagnosis. Although many cases of DEB-Pr are caused by mutations of type VII collagen due to reported variants in COL7A1 gene, the clinical pictures vary. We report a patient with DEB-Pr who presented with striking flagellate hypertrophic scarring lesions. Histopathological and electron microscopic findings are essential for diagnosis, given the considerable overlap with clinically similar presentations such as hypertrophic lichen planus, prurigo nodularis, lichen simplex chronicus and dermatitis artefacta. Case Presentation: Herein, we report a case with a history of blistering since childhood followed by intensely pruritic papulonodules, predominantly on the shins of tibia and dorsum of feet with distinct flagellate scarring pattern and toenail dystrophy. Our case was monitored before and after treatment with topical Tacrolimus 0.03% ointment and systemic Cyclosporine. Conclusion: Our case had an excellent response to topical Tacrolimus 0.03% ointment and systemic Cyclosporine treatment.