Clinical management for epidermolysis bullosa dystrophica

Thais M Oliveira, Vivien T Sakai, Liliani A Candido, Salete M B Silva, Maria Aparecida A M Machado, Thais M Oliveira, Vivien T Sakai, Liliani A Candido, Salete M B Silva, Maria Aparecida A M Machado

Abstract

Epidermolysis bullosa (EB) consists of a group of genetic hereditary disorders in which patients frequently present fragile skin and mucosa that form blisters following minor trauma. More than 20 subtypes of EB have been recognized in the literature. Specific genetic mutations are well characterized for most the different EB subtypes and variants. The most common oral manifestations of EB are painful blisters affecting all the oral surfaces. Dental treatment for patients with EB consists of palliative therapy for its oral manifestations along with typical restorative and periodontal procedures. The aim of this article is to describe two dental clinical treatments of recessive dystrophic EB cases and their specific clinical manifestations. The psychological intervention required during the dental treatment of these patients is also presented.

Figures

FIGURE 1. A= Frontal view of open…
FIGURE 1. A= Frontal view of open mouth with patient's help. The limitation of mouth opening was a consequence of microstomia. B = Maximal upper occlusal view
FIGURE 2. Panoramic radiograph showing the oral…
FIGURE 2. Panoramic radiograph showing the oral health status
FIGURE 3. A= Immediate (maxillary right central…
FIGURE 3. A= Immediate (maxillary right central and lateral incisors) and 7-day (maxillary left central and lateral incisors) postoperative aspect. Note the hemorrhagic blisters on the lip and palate after surgery. B= Oral aspect 1 month after the first surgical procedure
FIGURE 4. Frontal view of the patient's…
FIGURE 4. Frontal view of the patient's face partially showing the prosthetic appliance with two upper incisors
FIGURE 5. Frontal view of open mouth.…
FIGURE 5. Frontal view of open mouth. The limitation of mouth opening was a consequence of the microstomia
FIGURE 6. Panoramic radiograph showing the oral…
FIGURE 6. Panoramic radiograph showing the oral health status

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Source: PubMed

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