Epidermolysis bullosa: a case report

Abstract

Epidermolysis bullosa (EB), often referred to as the butterfly disease, is a group of rare genetic conditions characterized by skin that is delicate and fragile as butterfly wings. The skin blisters in response to friction, minor injury, or trauma. In certain types of EB, other organs, such as the esophagus, can also be affected, and secondary complications may require multiple interventions. While there has been significant progress in classifying the diseaseidentifying genes and proteins involved-there have been few advances in the treatment of the disease. The care of the EB patient focuses on management of symptoms, protecting the skin, and preventing complications. In this case report, the use of a multivalent wound-healing ointment (Terrasil®) was evaluated in a 60-year-old patient with a history of junctional EB. A polymerase chain reaction-based culturing was utilized to quantitatively test for bacteria and fungi at baseline and follow-up visits. Pain assessment and wound area were also documented at each visit. Following the application of the wound care ointment, there was a reduction in wound surface area on central (96%) and distal mid-back (92%) by treatment visit three, and there was a 96% reduction on the left shoulder blade ulcer by treatment visit four. Moreover, there was a noticeable drop in the percentage of bacteria detected by polymerase chain reaction. The wound care ointment was also effective in eliminating the fungal species and reducing pain, itching, blistering, and cracking around the wound.

Longitudinal study of wound healing status and bacterial colonisation of Staphylococcus aureus and Corynebacterium diphtheriae in epidermolysis bullosa patients

“…To our knowledge, this is the first report showing the specific presence of CD in Epidermolysis bullosa. Previous reports in EB patients have shown presence of other Corynebacterium species from wound cultures, such as C. striatum , C. simulans , C. tuberculostearicum or only reporting presence of Corynebacterium species (or spp) 19,33 . Interestingly, the interaction between Corynebacterium species and SA in non‐EB conditions has been shown to reduce SA virulence by shifting SA towards commensalism in response to Corynebacterium species 34 .…”

“…Previous reports in EB patients have shown presence of other Corynebacterium species from wound cultures, such as C. striatum , C. simulans , C. tuberculostearicum or only reporting presence of Corynebacterium species (or spp). 19 , 33 Interestingly, the interaction between Corynebacterium species and SA in non‐EB conditions has been shown to reduce SA virulence by shifting SA towards commensalism in response to Corynebacterium species. 34 How CD interacts and ultimately influences SA behaviour in EB is largely unknown but warrants further investigation.…”

Occam's Razor Versus Hickam's Dictum: A Case Report of Junctional Epidermolysis Bullosa and Lower Urinary Tract Infection

“…Numerous macromolecules that are necessary for the cohesiveness of the skin and its resistance to shearing forces have come to light in recent studies. It has expanded our understanding of the genetic changes and distinctive ultrastructural morphology that cause skin blistering [ 5 ].…”

“…Clinical observation can frequently lead to a provisional diagnosis, but histological examination of a skin sample yields the most conclusive results in the diagnosis of EB. Transmission electron microscopy, antigenic mapping, and immunofluorescence (IF) are essential diagnostic techniques for confirming and identifying a specific EB subtype [ 5 ]. The subtype of the illness can have an impact on its prognosis.…”

Epidermolysis bullosa with clinical manifestations of sepsis and pneumonia: A case report

“…He responded positively to caring crème for the wounds and dietary supplements for several months. EB was diagnosed based on clinical and histopathological tests of the skin and skeletal muscles [8] .…”