29 April 2025

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A Simple Method for Prosthodontic Rehabilitation of Edentulous Patient with Epidermolysis Bullosa: A Clinical Case Report

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An abnormally small oral orifice is defined as microstomia. Microstomia may result from epidermolysis bullosa (EB), which consists of a group of disorders characterized by the presence of mechanical fragility of the skin with recurrent de-velopment of blisters and vesicles, resulting from minor mechanical friction or trauma. Since such patients have a small oral aperture, it may be impossible to take impression and fabricate dentures using conventional methods. In this article, a simple method for taking preliminary impressions from upper and lower edentulous ridges in one patient with limited mouth opening and then preparing the complete denture with custom denture teeth in a single unit was described.

Introduction

An abnormally small oral orifice is referred to as microstomia. Microstomia may result from surgical treatment of orofacial neoplasms, cleft lips, maxillofacial traumas, burns, radiotherapy, scleroderma or genetic disorders such as epidermolysis bullosa and connective tissue diseases such as systemic sclerosis. Fragility and skin blistering are the hallmark features of the hereditary disorders classified as epidermolysis bullosa (EB). Although the specific pathogenesis of these disorders remains unknown, bulla formation has been associated with numerous basic defects including structural or biochemical abnormalities of keratin, hemidesmosomes, anchoring fibrils, anchoring filaments, and physicochemically-altered skin collagenase.

As many as 23 EB variants are recognized and delineated based on their clinical appearance, extra coetaneous involvement, mode of inheritance, and level of tissue cleavage. These subtypes are in turn classified into 3 main groups based on the level of tissue separation that develops after mechanical trauma to the skin. Blistering occurs within the epidermis, within the basement membrane, or beneath the basement membrane in simplex, junctional, and dystrophic forms of inherited EB, respectively. Systemic features include bulla formation on hands, feet, elbows, and knees. The lesions are initially noted at or soon after birth. Bulla leave painful ulcers and, rupturing and healing are often followed by scarring and tissue contraction. Contractures and syndactyly of digits might result in the formation of claw-like hands. The upper esophagus frequently becomes stenotic, leading to dysphagia or esophageal obstruction.

Oral features include repeated blistering and scar formation, leading to limited oral opening, ankyloglossia, and elimination of buccal and vestibular sulci, perioral structure, severe periodontal disease and alveolar bone resorption, atrophy of the maxilla with mandibular prognathism, an increased mandibular angle, and predisposition to oral carcinoma. Routine dental care or even normal tooth brushing might cause bulla on the oral mucosa. Painful blisters, restricted oral opening and poor manual dexterity as a result of finger deformities are factors contributing to diminished oral hygiene and delayed dental maturity. Satisfactory dental management of patients with EB represents one of the most difficult clinical challenges for the dentist as oral condition of these patients results in poor residual dentition and may lead to partial or complete edentulism.

Prosthetic rehabilitation of microstomia patients presents difficulties at all stages, from the preliminary impressions to fabrication of prostheses.9 Several techniques in patients with microstomia based on flexible, modified standard trays, sectioned trays, sectional , and collapsible dentures have been proposed. In this clinical report, another simple method is presented, describing all stages from preliminary impressions to fabrication of complete dentures in single unit lined with laboratory soft liner, for a patient with limited mouth opening resulting from EB.

Authors: Farhang Mahboub, Katayoun Sadr, Fateme Heidary, Elham Hosseini 

Source: https://pmc.ncbi.nlm.nih.gov/