Epidermolysis bullosa pruriginosa: A report of two cases
A 52-year-old lady presented to our outpatient department with complaints of itching and blackish discoloration of skin of both the lower limbs for more than 35 years and fluid filled lesions over the lower limbs since two years. Lesions were extremely pruritic without any diurnal variation. There was no history of drug intake before the onset of lesions nor any seasonal exacerbation. There was no history of similar complaints in other family members. Patient was a known case of diabetes since five years and on regular treatment. On examination, multiple lichenified papules to nodules were present over the lower limbs extending from the knee to the ankle joint. Depigmentation at the center with scarring was seen over the larger nodules. Few pus filled lesions were also seen []. There was no associated nail involvement. Routine investigations were normal. Based on the clinical picture, EB pruriginosa was suspected. Skin biopsy and immunohistochemistry was done to confirm the diagnosis.\nOn histopathological examination, epidermis showed subepidermal and intraepidermal bulla with degeneration of keratinocytes. Foci of cellular infiltrate consisting of fragmented neutrophils with occasional mononuclear cells and hemorrhage [Figures and ]. Direct immunofluorescence was negative. Patient was admitted for four days and was given parenteral steroids and antibiotics. Itching subsided and lesions began to heal. On discharge patient was started on tab dapsone 100 mg once a day with good response. Hematological parameters and lipid profile were monitored.