enamel hypoplasia ( eh ) is a defect in tooth enamel that results in less quantity of enamel than normal . the defect can be a small pit or dent in the tooth or can be so widespread that the entire tooth is small and/or malformed . this type of defect may cause tooth sensitivity , may be unsightly or may be more susceptible to dental caries it can appear white , yellow or brownish in color with a rough or pitted surface . in some cases , environmental and genetic factors that interfere with tooth formation are thought to be responsible for eh . this includes trauma to the teeth and jaws , intubation of premature infants , infections during pregnancy or infancy , poor pre - natal and post - natal nutrition , hypoxia , exposure to toxic chemicals and a variety of hereditary disorders . frequently , the cause of eh in a particular child is difficult to determine . this case report presents with two cases of amelogenesis imperfecta as an example of a hereditary disorder . a 14year - old female patient visited the department of periodontics at the oxford dental college and hospital with a chief complaint of discoloration of teeth . the patient reported that her teeth were at this state from the time of eruption and her deciduous teeth too had appeared the same . she was systemically healthy and gave a family history that revealed her mother and younger sister also had similar discoloration of teeth . her oral hygiene habits involved brushing once a day using a medium brush with the horizontal method of brushing . all teeth had yellowish discoloration with some patches of brown color in a few teeth . the patient had a class i malocclusion with a cross bite in relation to the first premolar on both sides . the occlusal surface of both the upper and lower molars showed severe attrition as a result of which the dentine was exposed . the second premolars of both the upper and lower jaws showed varying degrees of attrition [ figure 1a  e ] . gingiva appears moderately inflamed ( b ) moderate gingival inflammation in relation to the interdental papilla of upper and lower premolars , also note the crossbite between the upper and lower right first premolars ( c ) moderate gingival inflammation in relation to the interdental papilla of upper and lower premolars , also note the crossbite between upper and lower left first premolars ( d ) severe attrition in upper right and left first molars with dentine being exposed ( e ) severe attrition in lower first and second molars on both the sides with dentine being exposed ( f ) deep periodontal pocket measuring 8 mm in relation to mesial surface of 36 ( g ) deep periodontal pocket measuring 8 mm in relation to mesial surface of 46 ( h ) orthopantomogram showing pulp stones with most of the teeth , loss of cusp tips with respect to the upper and lower molars . bone loss in relation to the mesial surfaces of all the first molars , indicative of localised aggressive periodontitis ( i ) functional and esthetic rehabilitation of patient 's teeth with ceramic crowns the oral hygiene status of the patient was good with minimal plaque scores . there were deep periodontal pockets in relation to the mesial surface of all the first molars . there was no furcation involvement and mobility associated with these teeth [ figure 1f and g ] . an orthopantomogram and an intraoral periapical radiographs in relation to all the first molars were taken . the orthopantomogram revealed that the enamel was very thin generally and even absent in relation to the molars of both upper and lower jaw . all the molars showed the presence of pulpal calcifications in the pulp chamber [ figure 1h ] . a diagnosis of amelogenesis imperfecta and localised aggressive periodontitis were made after a detailed examination of the teeth and periodontium . the treatment involved scaling and root planing , followed by antibiotic therapy with 100 mg doxycycline ( loading dose 200 mg on the first day followed by 100 mg once a day for 13 days ) . bio - oss bone graft was placed in both the sites to attempt regeneration in relation to the intrabony defects in relation to these teeth . the probing depths in relation to the upper first molars reduced following non - surgical therapy and so further periodontal surgery was not needed . after 3 months of completion of the periodontal therapy , the patient 's teeth were restored with full ceramic crowns . the patient was on a regular maintenance programme and has been followed up for 3 years [ figure 1i ] . a 19-year - old female patient presented with discoloration and sensitivity of teeth . the teeth were discolored since eruption , and the deciduous teeth presented the same problem before they were exfoliated . the teeth were brown and pitted , with the enamel presenting a cheesy consistency in the cervical areas of many teeth . the gingiva was erythematous in all marginal and papillary areas with a soft and edematous consistency , with bleeding on probing in all areas . exudation was positive in the anterior and bicuspid areas of the maxillary and mandibular arches . the mandibular teeth did not show any significant probing depths [ figure 2a  c ] . ( a ) note the absence of enamel in the upper and lower teeth and complete absence of cuspal tips in the lower teeth due to severe attrition . ( b ) proximal caries in relation to mesial surfaces of both the upper lateral incisors . presence of deep periodontal pocket in relation to the distal surface of right central incisor ( c ) the gingiva appears moderately inflammed with bulbous interdental papillae and rolled margins . presence of deep periodontal pocket in relation to the distal surface of left central incisor on radiographic examination the teeth generally had a thin layer of enamel , in relation to the upper and lower molars the enamel was absent due to severe attrition . the lower left first molar presented with horizontal bone loss and the upper incisors and right first molar presented with vertical bone loss [ figure 3 ] . orthopantomogram shows proximal caries in relation to lower first molars and initial bone loss in relation to the distal surfaces of both molars based upon these clinical findings a diagnosis of localized aggressive periodontitis associated with amelogenesis imperfecta was given . for unknown reasons the patient , however , did not return for the treatment . a 14year - old female patient visited the department of periodontics at the oxford dental college and hospital with a chief complaint of discoloration of teeth . the patient reported that her teeth were at this state from the time of eruption and her deciduous teeth too had appeared the same . she was systemically healthy and gave a family history that revealed her mother and younger sister also had similar discoloration of teeth . her oral hygiene habits involved brushing once a day using a medium brush with the horizontal method of brushing . all teeth had yellowish discoloration with some patches of brown color in a few teeth . the patient had a class i malocclusion with a cross bite in relation to the first premolar on both sides . the occlusal surface of both the upper and lower molars showed severe attrition as a result of which the dentine was exposed . the second premolars of both the upper and lower jaws showed varying degrees of attrition [ figure 1a  e ] . gingiva appears moderately inflamed ( b ) moderate gingival inflammation in relation to the interdental papilla of upper and lower premolars , also note the crossbite between the upper and lower right first premolars ( c ) moderate gingival inflammation in relation to the interdental papilla of upper and lower premolars , also note the crossbite between upper and lower left first premolars ( d ) severe attrition in upper right and left first molars with dentine being exposed ( e ) severe attrition in lower first and second molars on both the sides with dentine being exposed ( f ) deep periodontal pocket measuring 8 mm in relation to mesial surface of 36 ( g ) deep periodontal pocket measuring 8 mm in relation to mesial surface of 46 ( h ) orthopantomogram showing pulp stones with most of the teeth , loss of cusp tips with respect to the upper and lower molars . bone loss in relation to the mesial surfaces of all the first molars , indicative of localised aggressive periodontitis ( i ) functional and esthetic rehabilitation of patient 's teeth with ceramic crowns the oral hygiene status of the patient was good with minimal plaque scores . there were deep periodontal pockets in relation to the mesial surface of all the first molars . there was no furcation involvement and mobility associated with these teeth [ figure 1f and g ] . an orthopantomogram and an intraoral periapical radiographs in relation to all the first molars were taken . the orthopantomogram revealed that the enamel was very thin generally and even absent in relation to the molars of both upper and lower jaw . all the molars showed the presence of pulpal calcifications in the pulp chamber [ figure 1h ] . a diagnosis of amelogenesis imperfecta and localised aggressive periodontitis were made after a detailed examination of the teeth and periodontium . the treatment involved scaling and root planing , followed by antibiotic therapy with 100 mg doxycycline ( loading dose 200 mg on the first day followed by 100 mg once a day for 13 days ) . bio - oss bone graft was placed in both the sites to attempt regeneration in relation to the intrabony defects in relation to these teeth . the probing depths in relation to the upper first molars reduced following non - surgical therapy and so further periodontal surgery was not needed . after 3 months of completion of the periodontal therapy , the patient 's teeth were restored with full ceramic crowns . the patient was on a regular maintenance programme and has been followed up for 3 years [ figure 1i ] . the teeth were discolored since eruption , and the deciduous teeth presented the same problem before they were exfoliated . the teeth were brown and pitted , with the enamel presenting a cheesy consistency in the cervical areas of many teeth . the gingiva was erythematous in all marginal and papillary areas with a soft and edematous consistency , with bleeding on probing in all areas . exudation was positive in the anterior and bicuspid areas of the maxillary and mandibular arches . the mandibular teeth did not show any significant probing depths [ figure 2a  c ] . ( a ) note the absence of enamel in the upper and lower teeth and complete absence of cuspal tips in the lower teeth due to severe attrition . ( b ) proximal caries in relation to mesial surfaces of both the upper lateral incisors . presence of deep periodontal pocket in relation to the distal surface of right central incisor ( c ) the gingiva appears moderately inflammed with bulbous interdental papillae and rolled margins . presence of deep periodontal pocket in relation to the distal surface of left central incisor on radiographic examination the teeth generally had a thin layer of enamel , in relation to the upper and lower molars the enamel was absent due to severe attrition . the lower left first molar presented with horizontal bone loss and the upper incisors and right first molar presented with vertical bone loss [ figure 3 ] . orthopantomogram shows proximal caries in relation to lower first molars and initial bone loss in relation to the distal surfaces of both molars based upon these clinical findings a diagnosis of localized aggressive periodontitis associated with amelogenesis imperfecta was given . for unknown reasons the patient , however , did not return for the treatment . amelogenesis imperfecta is a composite of hereditary disorders that primarily affects enamel of both deciduous and permanent teeth . studies have reported the prevalence of amelogenesis imperfecta to be 1 out of 700 to 1 out of 14000 depending on the population examined . many cases are inherited as autosomal dominant , autosomal recessive or as an x - linked trait . there can be three basic types namely hypoplastic ( 60 to 70% ) , hypomaturation type ( 20 to 40% ) , and hypocalcification type ( 7% ) . classification of amelogenesis imperfecta by witkop and sauk the clinical presentation depends on the type of amelogenesis imperfecta ; varying from the mild hypomaturation snow capped enamel to the more severe hereditary hypoplastic with thin , hard enamel , which has a yellow brown appearance . in the hypoplastic type , the teeth appear to be normally hard , although the enamel in the deeper portions may be poorly mineralized . schulze c recorded that the teeth could be yellow , yellowish red , or yellow - brown . radiographs exhibit a thin peripheral out line of radio dense enamel , and low or absent cusps . in the hypomaturation type , the affected teeth exhibit mottled , opaque white , brown - yellow discoloured enamel , which is softer than normal . in radiographs the thickness of enamel is normal but density is the same as that of dentine . radiographs show that enamel thickness is normal but its density is even lesser than that of dentine . in this report the first patient 's teeth were conformed to the description of hypoplastic type of amelogenesis imperfecta , both clinically and radiographically . the second patient 's teeth conferred to the description of hypomineralized type of amelogenesis imperfecta because of the cheesy consistency and ease with which the enamel could be detached . the other dental manifestations include pulpal calcification , taurodontism , root malformation , impaction of permanent teeth , progressive root and crown resorption , congenital missing teeth , and anterior open bite . the first patient 's radiograph showed presence of pulpal calcifications in most of the teeth . histologically there is a disturbance in the differentiation or visibility of ameloblasts in the hypoplastic type , and this is reflected in defects in matrix formation up to and includes total absence of matrix . in the hypocalcification type , there are defects of matrix structure and of mineral deposition . finally in the hypomaturation types the diagnosis is made by eliminating environmental factors like fluorosis , trauma , exanthematous fevers , hypocalcemia , or hypoplasia due to calcium deficiency as a possible cause , recognising the in heritance pattern , and phenotype . radiographs including full mouth intraoral periapical radiographs , orthopantomogram will play a vital role in diagnosing the difference in density of enamel in amelogenesis imperfecta and normal patients along with dentine thickness , density , pulp canals , and root length . treatment planning for patients with amelogenesis imperfecta is related to the age , socioeconomic factors , the type , and severity of the disorder and intraoral structures . an interdisciplinary approach may be required to evaluate , diagnose , and resolve the esthetic problem . as was done with the first case where the patient underwent prosthetic rehabilitation for esthetic and functional purpose . localised aggressive periododntitis previously known as localised juvenile periodontitis is one of the rapidly progressive periodontal diseases . it is characterised by pattern of rapid vertical loss of alveolar bone around more than one permanent tooth mainly the permanent first molars and incisors . the severity of the destruction is not proportional to the mass of plaque or calculus present . clinical picture of localised aggressive periodontitis is characterised by an insidious onset during the circumpubertal period . the most striking feature is that during the early stage there is lack of clinical sign of inflammation . there is severe periodontal destruction which is evidenced by deep periodontal pocket formation , tooth mobility , and pathological migration . in advanced stages diastema formation , sensitivity to thermal and tactile stimuli from denuded root surface may appear . radiographic findings show vertical bone loss in relation to the first molars and/or incisors . in the classic localised juvenile periodontitis ; an arc shape bone loss pattern is seen extending from the distal of the second premolar to the mesial of the second molar . the diagnosis is made based on clinical examination , radiographic appearance , and lack of systemic involvement . treatment of localised aggressive periodontitis involves the combination of scaling and root planing along with systemic antibiotic therapy followed by periodontal flap surgery in areas of persisting deep pockets . a linkage study on a dentinogenesis imperfecta - iii kindred with juvenile periodontitis ( jp ) has also suggested that this disease may be linked to the long arm ( q ) of human chromosome 4 . furthermore , the autosomal domininant local hypoplastic form of amelogenesis imperfecta has also been recently linked to a 15.2 cm region of human chromosome 4 in the region 4q11 - 4q13 . the proposal is based on the following hypotheses : that mutations within the human ameloblastin ( ambn ) gene localized to 4q21 causes the local hypoplastic form of adai . thus suggesting that there may be a genetic link between amelogenesis imperfecta and localized aggressive periodontitis , this however needs further investigation .
this case report presents two female patients whose chief complaint was discoloration of teeth . on careful clinical examination 
 it was found that the patients had features of amelogenesis imperfecta and localised aggressive periodontitis . 
 this article will give an insight of clinical and radiographic features of amelogenesis imperfecta with localised aggressive periodontitis , which is a rare clinical entity .