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Epidermolysis bullosa
Author(s) -
Wright J. T.,
Gantt D. G.
Publication year - 1983
Publication title -
journal of oral pathology and medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.887
H-Index - 83
eISSN - 1600-0714
pISSN - 0904-2512
DOI - 10.1111/j.1600-0714.1983.tb00320.x
Subject(s) - enamel paint , dentin , epidermolysis bullosa , molar , dentistry , medicine , amelogenesis imperfecta , epidermolysis bullosa simplex , cementum , anatomy , pathology
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.