308300 ) is a rare x - linked dominant genodermatosis characterized by typical skin lesions along blaschko s lines and associated with ocular , dental , nails , hair , skeletal , central nervous system and cardiovascular anomalies . dental abnormalities are most frequent and present in 60% of patients older than 1 year . partial anodontia of decidious or permanent teeth and peg - shaped or conical teeth are the most frequent dental problems [ 14 ] . some patients were reported to have multiple caries and  crumbly  teeth . in patients with incontinenti pigmentia and dental abnormalities , significant alterations in the mineralization of the teeth have also been observed . early caries and general poor dental quality may be specific for both first and second dentitions . the diagnosis of dental changes can not be given earlier than the age of one year . he was born to a 40-year - old mother , g2p2 , at full term after an uneventful pregnancy . the lesions then had cleared gradually leaving linear pigmentation . in physical examination , he was overweight and mentally retarded . hyperpigmented lines were noted along blaschko s lines in his trunk and limbs ( figure 1 ) . hypopigmented atrophic streaks first and second mandibular left primary molar teeth ( k , l ) were absent and in the maxilla , right first primary molar , right primary central incisor , left primary lateral incisor and left second primary molar ( b , e , g , j ) were missing . on oral examination of the patient , some of both mandibular and maxillary anterior teeth were conical and in the radiograph , the primary teeth roots were normal . the first right mandibular permanent molar ( 30 ) showed a delayed development compared to their maxillary counterparts . regarding susceptibility to caries , the teeth were normal and there was only occlusal caries in the first right primary mandibular molar tooth ( s ) which was treated by amalgam . the interdental spaces were visible due to some missing teeth , sub - mucosal cleft palate and the conical condition of some other teeth . radiography revealed 13 un - erupted permanent teeth , including six mandibular anterior teeth ( 22,23,24,25,26 and 27 ) ) , first right premolar ( 28 ) and molar ( 30 ) , four maxillary incisors and first left molar ( 6,7,8,9,10,11 and 14 ) . ip is an x linked dominantly inherited disorder reported primarily in females and believed to be embryonically lethal in the majority of males . in the majority of cases ip is due to a mutation in a gene called nemo ( nf-b essential modulator ) which normally protects against tnf - induced apoptosis . it is characterized by typical skin lesions along blaschko s lines usually evolving through four stages : 1-vesicular ( from birth or shortly thereafter ) ; 2-verrucous ( between 2 and 8 weeks of age ) ; 3-hyperpigmented ( several months of age into adulthood ) and 4-hypopigmented ( from infancy through adulthood ) . the inflammatory sequence sometimes recurs within the pigmented area during the first few months of life , particularly at the time of febrile illness . the epidermis of verrucous lesions is acanthotic with dyskeratosis and foci of dyskeratosis . in stage 3 , there is pigmentary incontinence ; whereas , stage 4 is characterized by a thinned epidermis and dermis devoid of appendages . associated cutaneous findings include patchy scarring alopecia , woolly hair nevi , nail dystrophy and anhydrosis in atrophic streaks.extracutaneous features include dental abnormalities ( partial anodontia and conical or peg - shaped teeth ) , cns abnormalities ( seizure , delayed psychomotor development , mr , spastic hemiplegia ) and ocular disease ( retinal vascular abnormalities , strabismus and blindness ) . therefore , all affected infants should be referred for a baseline ophthalmologic examination and periodic neurodevelopmental and dental evaluation . our patient showed the classical sequential cutaneous findings of ip ( vesicular , verrucous lesions and pigmentation along blaschko s lines as well as linear hypopigmented atrophic hairless lesions ) . although ip is mainly seen in females and generally lethal in males in utero , surviving males are exceptional . some male cases were due to concomitant klinefelter syndrome ( xxy ) , but in the majority , no such association was present . an outstanding feature in our patient was his characteristic abnormal dentition , for which the parents sought dentist consultation . in our case , dental examination showed partial hypodontia , peg - shaped teeth and delayed teeth eruption , typically seen in ip [ 14 ] . there were no signs of other abnormal findings including gothic plate that were reported by mini   and himelhoch et al . . unlike studies reporting hypocalcifications and prevalent caries , our patient had no enamel defects or susceptibility to caries and only minimal caries on the occlusal surface of the first primary right mandibular molar was detected . this patient was interesting for the presentation of ip in a boy as well as his characteristic dental findings .
incontinentia pigmenti ( ip ) is an x - linked dominant genodermatosis characterized by typical skin lesions along blaschko s lines and associated with ocular , dental , nails , hair , skeletal , central nervous system and cardiovascular anomalies . 
 we report a 5-year - old boy with cutaneous hyperpigmentation along blaschko s lines , atrophic streaks , strabismus and mental retardation . 
 he showed the characteristic abnormal dentition seen in ip as partial hypodontia , peg - shaped anterior teeth and un - erupted teeth . 
 the expression of ip in boys is exceptional as the disease is lethal in males .