CUTANEOUS AND EXTRA CUTANEOUS MANIFESTATIONS AND FAMILY HISTORY AMONG PATIENTS WITH EPIDERMOLYSIS BULLOSA IN MISRATA - LIBYA

Epidermolysis bullosa refers to a group of rare genetic disorders marked by recurrentblister formation and fragility of the skin and can affect any epithelial tissues in thebody. The aim of the paper to presents the cutaneous as well as the extracutaneousmanifestations of epidermolysis bullosa among Libyan patients, and highlights theassociation between extracutaneous manifestation and family history. A retrospectivesingle-center case-control study on patients diagnosed with epidermolysis bullosaregistered in the genodermatosis outpatient clinic of the dermatology department atMisrata Medical Center. Ethical approval was obtained. Data were collected frompatients' files into an excel sheet and then analyzed using SPSS software. Seventy-threepatients were included. Mean age was 5.7 years and male to female ratio is 1:1.6.The most common skin manifestations were bullae with erythema, crusts, and erosions.69.8% of patients (51 patients) developed extracutaneous manifestations, such as oralblisters, dental hypoplasia, nail dystrophy, alopecia, constipation, dysphagia, or cornealopacities. Among which, 42 patients have a positive family history of epidermolysisbullosa. The odds ratio is 2.6667 and the P-value is 0.0884. Although, 42 patients (75%)with a positive family history of epidermolysis bullosa have extracutaneousmanifestations. Statistical analysis did not show a strong association. Further studieswith a bigger sample size are required.