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Year : 2011  |  Volume : 22  |  Issue : 2  |  Page : 348-351
Prosthodontic management of anhidrotic ectodermal dysplasia

Department of Pedodontics and Preventive Dentistry, People's Dental Academy, Bhopal, Madhya Pradesh, India

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Date of Submission08-Dec-2009
Date of Decision02-Jun-2010
Date of Acceptance10-Nov-2010
Date of Web Publication27-Aug-2011


Ectodermal dysplasia is characterized by the absence or defects of two or more ectodermally derived structures. Anodontia or hypodontia is the most striking dental manifestation. In severe hypodontia, there is lack of alveolar development with consequent protrusion and eversion of the lips. Patients with anhidrotic forms suffer from heat intolerance due to lack of sweat glands and mild infections may lead to death in infancy from hyperthermia. A case of a 4-year-old child with anhidrotic ectodermal dysplasia with partial anodontia is presented. Dental, oral, and physical features were taken into consideration in diagnosis and treatment planning for this patient. Clinical management consisted of removable partial prosthesis in maxillary arch and complete denture prosthesis in mandibular arch. The main aim of the treatment was to improve psychological development and to promote better functioning of the stomatognathic system.

Keywords: Anhidrosis, anodontia, hypodontia, hypotrichosis

How to cite this article:
Gupta S, Tyagi P. Prosthodontic management of anhidrotic ectodermal dysplasia. Indian J Dent Res 2011;22:348-51

How to cite this URL:
Gupta S, Tyagi P. Prosthodontic management of anhidrotic ectodermal dysplasia. Indian J Dent Res [serial online] 2011 [cited 2019 Oct 14];22:348-51. Available from:

   Case Report Top

A 4-year-old male patient of Indian origin presented to the Department of Pediatric Dentistry due to lack of teeth and problems with speech and mastication.

The general medical history was noncontributory except that he had frequent episodes of high fever (108΀F), which were relieved with ice cold packs. An evaluation by pediatrician resulted in the diagnosis of anhidrotic ectodermal dysplasia with hypotrichosis and hypodontia. Family history revealed that he was the only son of parents with nonconsanguinous marriage.

Extraoral examination revealed the typical facial physiognomy of anhidrotic ectodermal dysplasia with prominent forehead and ears, protuberant lips and a saddle nose. The patient also presented with sparse scalp hair and missing eyebrows [Figure 1]. The skin appeared dry with hyper pigmentation around the eyes and mouth. A diminished lower facial height contributed to a senile facial expression.
Figure 1: Preoperative facial view showing characteristic physiognomy (sparse eyebrows, hair, reduced facial height, and thick lower lip

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Intraorally there was complete absence of mandibular primary teeth, atrophic alveolar ridge with normal appearance of alveolar mucosa, and maxillary arch showed the presence of conical incisor teeth and deciduous second molars [Figure 2]. The panoramic radiograph confirmed the absence of permanent tooth buds except for permanent central incisors and right permanent first molar and extreme underdevelopment of maxillary and mandibular arches[Figure 3].
Figure 2: Intraoral view showing absence of teeth in maxillary other than conical anterior teeth and deciduous second molars; and atrophied mandibular ridge with complete absence of teeth

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Figure 3: Panaromic radiograph showing absence of other primary and permanent teeth

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In order to improve the appearance, mastication, and speech, the child was provided with removable partial dentures in upper arch and complete dentures in lower arch[Figure 4]. Cast partial dentures were avoided because of the changes in oral structures due to growth of child. So, it was decided to change the maxillary and mandibular partial dentures every 6 months serving as interim prosthesis. Other objectives of treatment regarding the dental condition were the preservation of bone, early development of normal mechanisms of chewing, speaking and swallowing, the establishment of normal facial characteristics and smile; the development of normal emotional and psychological profile and the fitting of a functional prosthesis with adequate retention, stability, and support [Figure 5] and [Figure 6].
Figure 4: Final prosthesis

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Figure 5: Intraoral view with complete rehabilitation of occlusion

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Figure 6: Postoperative facial view

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Behavior management techniques such as tell-show-do were used throughout the procedures for construction of the dentures. Primary tooth forms poured in soft self-cure acrylic were used for stress distribution and better esthetics. Denture base was fabricated using heat cure fiber reinforced acrylic material for stability and longevity. After the final insertion routine, oral hygiene instructions for dentures were given to the parents.

   Discussion Top

Ectodermal dysplasias are a group of hereditary disorders involving an absence or deficiency of tissues and structures derived from the embryonic ectoderm. Clinical signs include trichodysplasia (abnormal hair) in 91% of cases, tooth agenesis in 80%, onychodysplasia (abnormal nails) in 75%, and dyshidrosis (abnormal sweat glands) in 42%. [1] EDs are rare and incidence has been estimated at 1 in 10,000 and 1 in 100,000 births. [2] ED follows all possible modes of Mendelian inheritance, i.e., autosomal dominant or recessive or X-linked dominant or recessive and there have also been sporadic cases reported. Now there are over 170 different, clinically distinct subtypes. [3] Pathogenesis is thought to involve an altered epithelium-mesenchymal interaction. About 30 causative genes have been isolated. These are involved in intracellular communication and signaling, cell development, cell survival, and differentiation among other things.

Two different forms are clinically described, an autosomal inherited hydrotic form (Clouston's syndrome) and an X-linked anhidrotic form (Christ-siemens Touraine syndrome). [4] The anhidrotic form exhibits the classical triad and usually shows X-linked recessive inheritance severely affecting males, while females show only minor defects. In the hydrotic form teeth, hair and nails are affected. The sweat glands are usually spared giving normal appearance to the skin.

The anhidrotic form is commonly diagnosed in childhood, subsequent to a feverish episode of unknown origin. Palms and soles are hyperkeratotic; pseudorrhages are present around the eyes. Atopic eczema is common, especially in flexures during early childhood. In a newborn, a "plastic wrap" appearance of the skin is there. Partial or complete absence of sweat glands is responsible for the inability to perspire and accounts for intolerance to heat and hyperthermia. Sparse blonde hair including reduced density of eyebrow and eyelash hair is common. Clinical and radiological dental findings usually reveal multiple congenitally missing primary teeth, conoid primary incisors, and moderate to severe taurodontic second primary molars. The permanent teeth are always reduced in number.

In the present report, anhidrotic ectodermal dysplasia was suspected due to history of frequent episodes of high fever and senile appearance of the child. There is no specific treatment to minimize the relative consequences of the anomalies of fingernails, skin, and hair. Regarding the dental treatment instituted, it was possible to improve the physical condition of the child, as well as the esthetics, chewing, and phonetic functions. Treatment generally includes a removable or fixed partial denture, a complete overdenture prosthesis (overlaying affected teeth when the vertical dimensions of occlusion permits), and an implant retained prosthesis when indicated. These treatment approaches can be used individually or in combination to provide an optimal result. The proper sequencing of treatment is important to achieve the desired function and esthetic results.

In this case, the removal of the conoid teeth for the subsequent placement of the prosthetic appliance would reduce the alveolar ridge of the patient. Therefore, the maintenance of these teeth would promote a larger area for distribution of the chewing forces and greater retention of the prosthesis. The teeth were preserved without need for restoration and the child showed great cooperation and was satisfied with the esthetics.

Oral rehabilitation of ED has historically evolved from partial and complete removable prosthesis supported by tissue and teeth to screw-type osseointegrated dental implants. Guckes, Mccarthy, and Brahmin [5] claimed that implants placed in ED patients younger than 18 years of age have a high risk of failure. Kearns and Sharma [6] reported submergence of implants placed in two young patients (5 and 7 years) who later required placement of longer abutments and prosthesis revisions to accommodate the change in implant positions due to growth. For the patient of this report, prosthetic rehabilitation instead of placement of implants was considered according to growth of the patient. The decision to commence implant therapy early in life is a complex one. The treatment guidelines of the National Foundation for ED state that implants are only recommended for the anterior portion of the mandible in children older than school age (7 years or older). [7] The disadvantage of changing abutment configurations and replacement of prosthesis should be weighed against the psychological benefits of the patient.

Periodic recall of the young patients with ectodermal dysplasia is also important because prosthetic modification or replacement will be needed as a result of continuing growth and development. Dental treatment as a phase in the management of ectodermal dysplasia is essential. The first goal of a prosthodontic device is to meet the immediate needs of the young patient. To obtain a successful outcome, there needs to be a good communication between the professionals in an interdisciplinary team and patients/caretakers.

   References Top

1.Holbrook KA. Structural Abnormalities of the epidermally derived appendages in skin from patients with ectodermal dysplasia: Insight into developmental errors. In: Salinas CF, Optiz JM, Paul NW, editors. Recent advances in Ectodermal dysplasia. New York: Alan R. Liss; 1988. p. 15-44.  Back to cited text no. 1
2.Newton FM, Pinheiro M. Ectodermal dysplasias - Some recollections and a classification. In: Salinas CF, Optiz JM, Paul NW, editors. Recent advances in Ectodermal dysplasia. New York: Alan R. Liss; 1988. p. 3-14.  Back to cited text no. 2
3.Lamartine J. Towards a new classification of ectodermal dysplasia. Clin Exp Dermatol 2003;28:351-5.  Back to cited text no. 3
4.Pinheiro M, Freire Maia N. Ectodermal Dysplasias: A clinical classification and casual review. Am J Med Genet 1994;53:153-62.  Back to cited text no. 4
5.Guckes AD, Mc Carthy GR, Roberts MW. Pattern of permanent teeth present in individuals with ectodermal dysplasia and severe hypodontia suggests treatment with dental implants. Pediatr Dent 1998;20:278-80.  Back to cited text no. 5
6.Kearns G, Sharma A, Perott D, Schmidt B, Kaban L, Vargervik K. Placement of endosseous implants in children and adolescents with hereditary ectodermal dysplasis. Oral surg Oral Med Oral Pathol Oral Radiol Endod 1999;88:5-10.  Back to cited text no. 6
7.Mascoutah, IL. Parameters of Oral health Care for individuals affected by Ectodermal Dysplasia syndromes.: National Foundation for Ectodermal Dysplasias; 2003. p. 1-28.  Back to cited text no. 7

Correspondence Address:
Shilpy Gupta
Department of Pedodontics and Preventive Dentistry, People's Dental Academy, Bhopal, Madhya Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0970-9290.84291

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  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]

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