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Paper text:
incontinentia pigmenti ( bloch sulzberger syndrome ) , a rare , hereditary syndrome inherited by x - linked dominant gene , primarily affects female infants and is lethal in males .
the ip gene has been mapped to xq28 , which encodes nuclear factor b essential modulator ( nemo ) .
the clinical manifestations include vesicular , verrucous , and peculiar swirled , pigmented cutaneous lesions sequentially .
associated findings include developmental defects of eyes ( cataract , uveitis , optic atrophy , strabismus , retrolental fibroplasia ) , teeth ( delayed dentition , partial anodontia , cone- or peg - shaped teeth , or absence of teeth ) , skeletal system ( skull and palatal defects ) , and central nervous system ( epilepsy , microcephaly , mental retardation , and slow motor development ) .
cutaneous lesions may also be accompanied by defects of cutaneous appendages in the form of cicatricial alopecia or small and dystrophic nails .
lesions may be seen either at birth or before the end of first week . rarely , skin lesions start appearing after first 2 months of birth .
brownish , bluish - grey , or slaty pigmentation in characteristically diagnostic , bizarre , splashed or  chinese figure distribution may persist for many years .
diagnostic criteria for incontinentia pigmenti include : major criteria : ( 1 ) typical neonatal vesicular rash with eosinophilia ; ( 2 ) typical blaschkoid hyperpigmentation on the trunk , fading in adolescence ; and ( 3 ) linear , atrophic hairless lesions .
minor criteria : ( 1 ) dental anomalies , ( 2 ) alopecia , ( 3 ) wooly hair , and ( 4 ) abnormal nails . with a definitive family history ,
the presence of any major criterion strongly supports the diagnosis of incontinentia pigmenti . in the absence of a family history
a 5-years - old muslim male child presented with cutaneous hyperpigmentation on trunk , extremities , and face .
the boy was an only child , born to third degree consanguineous parents with full - term normal delivery .
as informed by mother , the skin lesions started appearing at 1 week of age in the form of vesicular lesions on the lower extremities , which gradually spread onto the trunk .
the child had slate brown hyperpigmented macules on face and lower limbs in linear distribution .
widely distributed areas of pigmentation in whorled pattern were seen on trunk [ figure 1 ] .
his developmental milestones were delayed for age , and he had complaints of primary nocturnal enueresis .
systemic examination including ocular , central nervous system ( cns ) , and skeletal systems revealed nothing abnormal .
whorled pattern of hyperpigmentation on trunk in case 1 hypodontia with peg - shaped teeth in case 1 histopathological examination revealed mild epidermal hyperplasia ( acanthotic ) with dyskeratotic cells with few whorls of squamous cells with central keratinization .
a 20-days - old male child , second in order , born to non - consanguineous parents , with an uneventful normal vaginal delivery , was brought with the complaints of vesiculobullous eruptions on both lower limbs [ figures 3 ] and 4 ] and right arm since birth .
the other sibling was a male child who expired at 7 months of gestational age due to hydrops fetalis .
the infectious causes were excluded ( tzanck test showed no giant cells , and test for antibodies for hsv , vzv was negative ) . systemic examination including that of eyes , cns , and skeletal system revealed nothing abnormal .
routine blood , urine , and stool examinations did not show any abnormality , except for raised eosinophils on differential leukocyte count .
vdrl of mother and the child was non - reactive , and a skin biopsy was performed , which revealed spongiotic intra - epidermal vesicles with a rich eosinophil infiltrate [ figure 5 ] .
vesicular lesions on lower limbs in case 2 vesicular lesions on lower limbs in case 2 histopathology showing eosinophilic spongiosis and acanthosis in high power
a 5-years - old muslim male child presented with cutaneous hyperpigmentation on trunk , extremities , and face .
the boy was an only child , born to third degree consanguineous parents with full - term normal delivery .
as informed by mother , the skin lesions started appearing at 1 week of age in the form of vesicular lesions on the lower extremities , which gradually spread onto the trunk .
the child had slate brown hyperpigmented macules on face and lower limbs in linear distribution .
widely distributed areas of pigmentation in whorled pattern were seen on trunk [ figure 1 ] .
his developmental milestones were delayed for age , and he had complaints of primary nocturnal enueresis .
systemic examination including ocular , central nervous system ( cns ) , and skeletal systems revealed nothing abnormal .
whorled pattern of hyperpigmentation on trunk in case 1 hypodontia with peg - shaped teeth in case 1 histopathological examination revealed mild epidermal hyperplasia ( acanthotic ) with dyskeratotic cells with few whorls of squamous cells with central keratinization .
a 20-days - old male child , second in order , born to non - consanguineous parents , with an uneventful normal vaginal delivery , was brought with the complaints of vesiculobullous eruptions on both lower limbs [ figures 3 ] and 4 ] and right arm since birth .
the other sibling was a male child who expired at 7 months of gestational age due to hydrops fetalis . on examination , the child had vesiculo - bullous lesions on both lower limbs .
the infectious causes were excluded ( tzanck test showed no giant cells , and test for antibodies for hsv , vzv was negative ) . systemic examination including that of eyes , cns , and skeletal system revealed nothing abnormal .
routine blood , urine , and stool examinations did not show any abnormality , except for raised eosinophils on differential leukocyte count .
vdrl of mother and the child was non - reactive , and a skin biopsy was performed , which revealed spongiotic intra - epidermal vesicles with a rich eosinophil infiltrate [ figure 5 ] .
vesicular lesions on lower limbs in case 2 vesicular lesions on lower limbs in case 2 histopathology showing eosinophilic spongiosis and acanthosis in high power
therefore , the role of dermatologists and pediatrician is crucial in early diagnosis of ip .
identification of male patients is valuable because of their rarity . in our case , both patients had characteristic clinical features of the disease but were otherwise normal , which shows that both were suffering from mild variant of the disease , which belies the fact that the disease is lethal in males .
case 1 showed characteristic cutaneous whorled pigmentation with peg - shaped teeth and cvs abnormality .
this disease being incurable , genetic counseling is an important aspect of management , which helps to reduce the disease burden on the family and the society .
there is no specific test for incontinentia pigmenti , although genetic testing and dna analysis can detect the genetic disorder on the x chromosome .
we report 2 cases of incontinentia pigmenti in males who exhibit mild variant of the disease and hence this article belies the fact that this disease is fatal in males .
incontinentia pigmenti is an x - linked dominant disorder , which is fatal in males , and majority of cases reported are in females . here , we report 2 cases of males with incontinentia pigmenti . 
 case 1 has progressed through the classical stages of ip , giving history of vesicular and verrucous lesions and has presented with hyperpigmentation in whorled pattern . 
 case 2 has vesicular lesions in a linear distribution on the legs along with classical histopathological findings .