Ectodermal Dysplasia, a Case Report: a Challenge for Prosthodontic Solution

Introduction: Ectodermal dysplasia (ED) is an inherited disruption of two or more tissues and structures of ectodermal origin during their embryonal development. Hypodontia or anodontia of the primary and permanent teeth, poorly developed alveolar ridges and inappropriate мaxillo-mandibular relations are the most common oral manifestations. Management of patients with ED (depending on the phenotypic characteristics) needs a multidisciplinary team approach that involves maxillo-facial surgeon, pedodontist, orthodontist and prosthodontist. Case report: A 6-year-old girl with ectodermal dysplasia having a normal intellectual development has typical facial expresion: square forehead, prominent ears and supraorbital ridges, pigmented and wrinkled eyelids, nose with anteverted nostrils, thin inward lips, and pointed chin. Hypotrichosis is characterized by almost no eyebrows, eyelashes and skin hairs; the sculp is covered with a wig. The skin is dry, thin and rough as a result of decreased function of the sweat glands. Finger and toe nails are short, thick and striated. Decreased lower facial height, together with deep mentolabial and nasolabial folds contributed to an old-looking facial expression. Complete bilateral cleft palate was partially solved on 1.5 year of age, with remained oro-nasal communication and a presence of soft palate only. An alveolar ridge in the mandible is rather atrophic with deciduous canines destroyed by caries and and carious permanent molar teeth in both jaws (severe hypodontia). The gingiva is rather swollen, with dark-red color especially around the teeth. Treatment procedure: Taking into consideration the patient’s age and clinical findings, removable complete over-denture prosthesis was the treatment of choice. The preliminary impressions were taken with stock trays and irreversible hydrocolloid material, while the functional impressions were taken using custom trays and thermoplastic material for border molding and polyvinyl siloxane impression material. Maxillo-mandibular relations were determined using temporary bases with wax rims. The final trial with artificial teeth arranged according to a balanced occlusion was conducted to verify horizontal and vertical relations, phonetics and esthetics. The maxillar and mandibular prosthesis were produced in the heat cure acrylic resin. Disrupted functions of the oro-facial system such as mastication, swallowing and phonetics are improved significantly with complete dentures. Conclusion: Correct maxillo - mandibular relations and normal function of the dentofacial system in patients with ED, can be achieved by the acrylic dentures. Prosthodontic treatment has a major impact not only on the functions and esthetics, but on the psychological development and emotional state as well. Keywords Cleft palate, Ectodermal Dysplasia, Hypodontia, Removable dentures

Role of humoral mechanisms in etiology of lichen planus

Aim: To examine the role of IgA, CIC and component C3 as indicators of humoral immune response in the etiopathogenesis of oral erosive lichen planus (OELP). Material and method: The study comprised 19 patients with OELP whose samples of blood, saliva and tissue were obtained after carefully taken medical history and clinical examination. Samples of oral mucosa were taken from the site of lesion, i.e. exclusively from buccal mucosa (1 cm in width and length), and from the deep epithelium as well as a segment from the lamina propria. Determination of immunoglobulins in serum and saliva, and determination of component C3, was done using the micro-elisa technique by Rook&Cameron, Engvall and Ulman. Determination of CIC in serum and mixed saliva was done with the PEG (polyethylene glycol) method. Determination of immunoglobulin A and component C3 in biopsy material was done with direct immunofluorescence. Results: Levels of immunoglobulin A in serum in OELP during exacerbation were decreased (1.04 ± 0.49 gr/l) and during remission increased (5.92 ± 0.62) in comparison with the control group (p < 0.001). Levels of CIC during exacerbation and remission were increased (p < 0.001), and component C3 levels were increased in both examined phases in the examined group compared with the control group (p < 0.05). Deposits of IgA were registered in one (5.88%) patient with OELP and component C3 was registered in 3 (17.64%) patients. Conclusion: Changes in IgA values, as well as CIC and component C3, may correlate with changes in oral mucosa emphasizing the role of humoral immune response in the pathogenesis of oral lichen planus