Epidermolysis bullosa dystrophica polydysplastica

Album, Manuel M.; Gaisin, Allen; Lee, K. W.Terry; Buck, Billy E.; Sharrar, William G.; Gill, Frances M.

doi:10.1016/0030-4220(77)90078-0

Cited by 16 publications

(3 citation statements)

References 24 publications

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“…The soft tissue manifestations of Dystrophic EB range from relatively mild to extremely severe 5, 24-27. For example, in Dominant Dystrophic EB the oral manifestations can involve an increased tissue fragility but infrequent blistering.…”

Section: Dermolytic Epidermolysis Bullosamentioning

confidence: 99%

Oral Manifestations in the Epidermolysis Bullosa Spectrum

Wright

2010

Dermatologic Clinics

107

122

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Section: Dermolytic Epidermolysis Bullosamentioning

confidence: 99%

Oral Manifestations in the Epidermolysis Bullosa Spectrum

Wright

2010

Dermatologic Clinics

107

122

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“…In the simplex form separation of tissue occurs in the epidermis during bullae formation. In the dystrophic form separation occurs subepidermally, causing damage to the elastic tissue , Album et al 1977. Epidermolysis bullosa may be further classified as either dominant or recessive (Cross et al 1968, Gorlin et al 1976).…”

mentioning

confidence: 99%

Epidermolysis bullosa

Wright

Gantt

1983

J Oral Pathology Medicine

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Epidermolysis bullosa (EB) describes a group of rare genetic mechanico‐bullous disorders. The disease has several modes of inheritance with various degrees of severity and expression. Two cases of recessive dystrophic EB are presented which show typical cutaneous, mucosal and occular involvement. The teeth were severely affected in both cases with much of the coronal enamel absent upon emergence into the oral cavity. The cervical enamel showed occasional hypoplastic pits. In one case, there was taurodontism of the permanent first molars. This case illustrates the increased occurrence of taurodontism associated with diseases affecting tissues of ectodermal origin. Taurodontism has not been previously reported with EB. Histologic studies of the teeth using light microscopy (LM) and scanning electron microscopy (SEM) revealed that the cementum and dentin were normal. The enamel, under LM, showed a prismatic structure with a broad neonatal line. SEM showed a wide amorphous zone of enamel adjacent to the dentin. Generalized throughout the enamel were globular areas within the prisms, which appeared to be local deformations. Hypoplastic pits on the surface were round and of varying diameters. The affected teeth exhibited both hypomaturation and hypo‐plastic defects of the enamel.

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“…Na epidermólise bolhosa distrófica, as manifestações nos tecidos bucais no tipo recessivo variam de moderada a extremamente grave (Crawford et al, 1976;Album et al, 1977;Wright et al, 1993). No tipo dominante, a fragilidade da mucosa bucal está presente, porém a formação de bolhas não é freqüente.…”

Section: Epidermólise Bolhosaunclassified

Necessidade de cuidados paliativos odontológicos em crianças com doenças crônicas. Caracterização de amostra

Carrillo¹

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Carrillo CM. Need of oral palliative care in children with chronic illness. Sample characterization [Dissertation]. São Paulo: Faculdade de Medicina, Universidade de São Paulo; 2011. INTRODUCTION: Epidermolysis bullosa is a genetic chronic disease that affects the quality of life deeply, whose oral symptoms compromised the daily living activities as chewing, swallowing and teeth brushing.. Children with cancer may also have oral manifestations, but transient and usually do not affect the teeth. This study assessed the oral health and the need of oral care in children in palliative care with epidermolysis bullosa compared to children with cancer. METHODS: Twenty-four patients with epidermolysis bullosa (Group A) and 18 cancer patients (Group B) followed up at the Pain and Palliative Care Outpatient Clinic were studied. Clinical assessment included: clinical characteristics, assessment of orofacial pain, oral health, quality of life according to the activities of daily living and general health status. RESULTS: Face numbness, tooth mobility, taste disturbance and speech difficulties were observed only in group B. Difficult chewing, disfagia, gagging, dry lips, oral lesions were more frequent in group A compared to group B, with significant difference, p<0,0001, p<0,0001, p=0,002, p<0,0001 and p<0,0001, respectively. Group A had a greater difficulty opening mouth, number of decay teeth, prevalence of caries and disability regarding the CHAQ eating (III) and reach (VII) domains than group B, with significant difference, p< 0,0001, p=0,0004, p< 0,001, p<0,001, p=0,005 and p<0,0001, respectively. Both groups showed a significant general health impairment. CONCLUSIONS: Epidermolysis bullosa patients in palliative care had chronic oral blistering and ulcerations, due to disease, more frequently than cancer patients, which had oral mucositis as a side effect of the antineoplasic treatment. They also showed greater oral symptoms, poor oral health, greater open mouth limitation and poor quality of life than cancer patients.

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Epidermolysis bullosa dystrophica polydysplastica

Cited by 16 publications

References 24 publications

Oral Manifestations in the Epidermolysis Bullosa Spectrum

Oral Manifestations in the Epidermolysis Bullosa Spectrum

Epidermolysis bullosa

Necessidade de cuidados paliativos odontológicos em crianças com doenças crônicas. Caracterização de amostra

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