A case report: the multiplex care of a patient with inherited epidermolysis bullosa

Abstract

Introduction. Inherited epidermolysis bullosa (EB) is a group of genodermatoses, characterized by mechanical fragility of the skin, blisters and erosions formation. The prevalence rate of EB is 2.4 cases/ 100,000 in Europe. Every year 1-2 babies with EB are born in Lithuania. Approximately 30 people could have EB in Lithuania. Clinical manifestations extent widely, from localized blistering of the hands and feet to generalized blistering of the skin and oral cavity, injury of many internal organs, impairment of a child development and growth. The management of the patients with different forms of EB is a challenge to make certain a maximum skin protection and not to add other comorbidities. Case description. A 33-year-old female with a known EB from the birth attended our department as her skin and common health status worsened as she became pregnant 17 weeks ago. Nutritional deficiency (anemia, hypoalbuminaemia, hypokalemia, dehydration), poor immunity and the risk of infection as big areas of the whole body were erosive and exudative, carcinogenic potentiality (the ulcer on the right knee) followed EB. The task of our staff was to provide an intensive care to improve the quality of life of the patient and to follow after the possible miscarriage. The multiplex care of the patient consisted of: 1) maintaining the highest possible level of nutrition and avoiding dietary complications; 2) prevention of infections; 3) analgesia; 4) wound care; 5) pregnancy saving because the patient wished to have another baby as the first girl was healthy. Also plastic surgeon, geneticist, obstetrition, psychiatrist consulted the patient. [...].