EPIDERMOLYSIS BULLOSA PRURIGINOSA, A CASE REPORT AND REVIEW OF THE LITERATURE

Abstract

Purpose: Epidermolysis bullosa pruriginosa (EBP) is a rare clinical subtype of inherited Epidermolysis bullosa dystrophica (EBD). It is characterized by severe itching and hypertrophic papules affecting the extensor surfaces of the extremities. Materials and methods: We present a 48 years old woman complaining about intense itching and plaques on the shins, started after mosquito bites. The patient was diagnosed with EBD, by electronic microscopy at the age of 32, because of blisters and painful erosions of the skin, appearing after trauma since birth. She has a daughter with the same skin disease. Results: Pathological skin changes were presented by excoriated erythematous papules and plaques on the lower 2/3 part of the shins. Erythemato-crustous lesions, atrophic scars, hypopigmented macules and milia in the elbows and knees were revealed. Severe toes nail dystrophy was found. The mucous membrane found no pathological changes. The complete blood count and biochemistry were in normal ranges. The microbiological examination found Staphylococcus aureus. The genealogical analysis revealed autosomal-dominant inheritance of the disease. The histopathological examination revealed a large subepidermal blister. On the basis of medical history, status and the results of histopathological and electronic microscopy examinations, the diagnosis EBP was confirmed. Treatment with desloratadine, emollients and topical corticosteroids was started. The patient is being followed up. Conclusion: We presented a rare case of EBP with autosomal-dominant inheritance in a 48 years old woman with the beginning of the pruritus and aggravation of clinical manifestation of the disease after mosquito bites.

Keywords

• epidermolysis bullosa pruriginosa
• autosomal-dominant inheritance
• mosquito bite