Does there exist a steroid responsive inflammatory variant of dystrophic epidermolysis bullosa? - A case report

A six-year-old girl presented with extensive blisters, erosions and scarring over trauma prone areas which started soon after birth. The lesions were refractory to conservative treatment with antibiotics and dressings. Peripheral blood count showed eosinphilia and raised ESR. Histopathology showed subepidermal bulla with prominent eosinophilic and lymphocytic infiltrate in dermis. Direct and indirect immunofluorescence tests were negative proving our clinical diagnosis of dystrophic epidermolysis bullosa. We gave a trial of oral corticosteroid and patient's condition improved dramatically. She once again started developing new lesions once we tapered the drug. We propose an existence of steroid responsive inflammatory variant of epidermolysis bullosa; an observation which is supported by many earlier published evidences.