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Giornale Italiano di Dermatologia e Venereologia 2014 December;149(6):651-62


lingua: Inglese

Inherited epidermolysis bullosa

El Hachem M., Giancristoforo S., Diociaiuti A.

Pediatric Dermatology Unit, Bambino Gesù Children’s Hospital, IRCCS, Rome, Italy


Inherited epidermolysis bullosa (EB) refers to a clinically and genetically heterogeneous group of rare disorders characterized by fragility of the skin and mucous membranes. Despite the preclinical development of different molecular and cell-based treatment strategies, no cure is still available for EB. Therefore it is important to establish the management of EB patient since neonatal age in order to ensure an early diagnosis and contribute to prevent complications and to improve quality of life. A review of literature was performed underlining the specific attention to coordinated multidisciplinary approach. The major topics treated comprise the multidisciplinary approach to EB patients, global skin care including wound care, management of itching and pain, and early diagnosis of squamous cell carcinoma. The rarity of EB and the variable involvement of several organs and systems challenge the appropriate treatment of these patients. The care of EB requires a coordinated multidisciplinary approach. The team should be specifically trained and a regular follow-up is required to prevent cutaneous and systemic complications. A well-organized and structured continuity of care is important in EB like in all chronic and rare diseases. The management of EB patients is very important because a specific attention to the fragile skin is required in order to reduce pain, risk of trauma, ulceration and infection. The center of expertise stays as the main structure offering specialized care to EB patients and also helps patients and their families to understand and deal with the disease and to ensure an adequate liaison with the community healthcare system.

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