Oropharyngeal lesions and their management in epidermolysis bullosa

Nowak, A. J.

doi:10.1001/archderm.124.5.742

Cited by 17 publications

(20 citation statements)

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“…Similar findings have been previously noted in individuals with generalized forms of recessive dystrophic EB [Wright, 19901. Increased caries in Hallopeau-Siemens EB is thought to occur secondarily to the soft tissue involvement [Wright et al, 19931. Severe soft tissue manifestations can lead to alteration of factors such as diet, oral retention time offood, and oral hygiene practices [Nowak, 1988;Wright et al, 19931. Although not specifically tested in this investigation, it seems likely that these same elements are responsible for an increased caries experience in EBDR-I.…”

Section: Discussionmentioning

confidence: 99%

Oral involvement of recessive dystrophic epidermolysis bullosa inversa

et al. 1993

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The inversa subtype of autosomal recessive dystrophic epidermolysis bullosa (EBDR-I) is a rare variant characterized by lesions involving primarily the flexural areas of the body. The purpose of this investigation was to characterize the oral manifestations of this unusual dermatologic condition. Ten individuals having EBDR-I were evaluated and compared with an age and sex-matched population of unaffected individuals that served as controls. The diagnosis of EBDR-I was confirmed by skin biopsy that demonstrated tissue separation below the lamina densa and the clinical presentation of blister formation that typically localized to flexural areas. There was clinical variability in the severity and distribution of skin involvement; however, none of the affected individuals demonstrated pronounced digital webbing, severe generalized blistering or growth retardation characteristic of the Hallopeau-Siemens form of EBDR. Oral involvement was seen in all cases with ankyloglossia, loss of tongue papillae and obliteration of the oral vestibule between the lips and gingiva being typical. The oral opening was significantly reduced in older EBDR-I individuals compared with matched controls, confirming that acquired microstomia is a characteristic of EBDR-I. The teeth were not clinically abnormal or malformed and showed no evidence of generalized enamel hypoplasia. Despite this, the prevalence of dental caries in EBDR-I individuals was significantly higher than the control group. The inversa form of EBDR presents with oral findings that are similar but generally milder than those seen in the Hallopeau-Siemens variant of EBDR.

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Section: Discussionmentioning

confidence: 99%

Oral involvement of recessive dystrophic epidermolysis bullosa inversa

et al. 1993

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“…It has been hypothesized that excessive caries is a result of the presence and severity of the soft tissue involvement, which leads to alteration in diet (soft and frequently carbohydrate foods); increased oral clearance time (secondary to limited tongue mobility and vestibular constriction); creation of an abnormal tooth/soft tissue relationship (i.e., buccal and lingual mucosa, which is firmly positioned against the tooth); and prevents the practice of normal oral hygiene measures. Furthermore, enamel hypoplasia that favors the retention of food debris decreases the tooth's intrinsic disease resistance (9,10).…”

Section: Discussionmentioning

confidence: 99%

Localized Epidermolysis Bullosa Simplex with Generalized Enamel Hypoplasia in a Child

Cagirankaya¹,

Hatipoğlu²,

Hatipoğlu

2006

Pediatric Dermatology

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Epidermolysis bullosa is an uncommon disease characterized by the formation of blisters following minor trauma. The three major types are simplex, junctional, and dystrophic. Generalized enamel hypoplasia is accepted as a feature of the junctional type. Teeth with enamel hypoplasia are liable to destruction in the form of caries and/or gingivitis. In this report, a patient with epidermolysis bullosa simplex and severe enamel hypoplasia is described and the importance of early dental consultation for all children with this disease is stressed.

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“…A extração dentária deve ser evitada nos pacientes com formas graves, pois reabilitações protéticas podem injuriar os tecidos bucais. Da mesma forma, dispositivos ortodônticos favorecem o atrito com tecidos bucais, a formação de bolhas e infecções 5,13,16 .…”

Section: Manifestações Bucaisunclassified

Atendimento multidisciplinar do paciente ortodôntico com epidermólise bolhosa

Alves

Alves²,

Maciel

et al. 2007

Rev. Dent. Press Ortodon. Ortop. Facial

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O termo epidermólise bolhosa descreve um grupo variado de doenças hereditárias, crônicas, não inflamatórias, epiteliais e da mucosa, que são caracterizadas por excepcional fragilidade e reduzida resistência após trauma moderado. Esta doença é classificada em forma simples, juncional ou distrófica, com pelo menos 23 subtipos, e é uma doença rara que afeta toda a população, qualquer grupo racial e igualmente homens e mulheres. OBJETIVO: os autores relatam problemas sistêmicos e bucais associados à epidermólise bolhosa, norteando atitudes clínicas multidisciplinares quando o paciente necessita de tratamento ortodôntico.

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Oropharyngeal lesions and their management in epidermolysis bullosa

Cited by 17 publications

References 0 publications

Oral involvement of recessive dystrophic epidermolysis bullosa inversa

Oral involvement of recessive dystrophic epidermolysis bullosa inversa

Localized Epidermolysis Bullosa Simplex with Generalized Enamel Hypoplasia in a Child

Atendimento multidisciplinar do paciente ortodôntico com epidermólise bolhosa

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