INTRODUCTION: Epidermolysisbullosa refers to group of inherited disorders involving formation of blisters following trivial trauma. Epidermolysis bullosa pruriginosa is a type of dystrophic epidermolysis bullosa caused by type VII collagen gene COL7A1 mutation, characterized by pruritus, nodular prurigo like lichenified lesions in early phase and violaceous linear scarring, trauma induced blistering, excoriation, milia, nail dystrophy in late healing phase, in some cases albopapuloid lesions can be seen on the trunk.

CASE SUMMARY: 27 years old male had an itchy, discrete, multiple papular and lichenified nodular lesions of varying sizes from 0.5–1.5cm, present over legs since the age of 12. No history of blistering after trauma, nail or mucosal involvement. No history of consanguineous marriage. His father and mother had similar lesions over the extremities. Histo–pathological examination of skin biopsy showed sub–epidermal blister with thickened stratum corneum in roof of blister. Within the blister, fibrin, plasma and few neutrophils were seen. Overlying epidermis showed mild acanthosis, focal thinning and lamellated compact orthohyperkeratosis. Papillary dermis thickened & showed abundant fibroplasia with increased number of thick walled capillaries & moderately dense mixed inflammatory infiltrate. Clinical features & biopsy findings suggestive of epidermolysisbullosa pruriginosa. Treatment with topical super–potent steroids and systemic antihistamine showed no response after 1 month.

CONCLUSION: Epedermolysis bullosa pruriginosa is a type of dystrophic Epidermolysis bullosa caused by type VII collagen gene COL7A1 mutation. It can be complicated by infection, deformities, squamous cell carcinoma which may metastasize eventually leading to death. Multidisciplinary approach with avoidance of trauma is required. Genetic counselling of parents is advisable.