Incontinentia Pigmenti: A Rare Genodermatosis in a Male Child

Case RepoRtAn 8-day-old neonate, second male child of nonconsanguineous parents, born out of an uneventful normal vaginal delivery, was brought with the complaints of erythematous papular skin lesions which was initially vesicular all over the body since birth.Mother had no history of miscarriage in the past and her 1 st child was a 3-yearold healthy female.On examination, the child had papular lesion on an erythematous base that follows the Blaschko lines over the abdomen, face and trunk [Table /Fig- 1] suggestive of Incontinentia pigmenti stage 1 (Vesicular stage).Hair and nails were normal.Systemic examination including that of eyes (Fundus), Central Nervous System, and skeletal system revealed no abnormalities.Complete blood count showed raised eosinophils on differential leukocyte count.At 1month follow up the child showed wide spread area of verrucous whorled papules on erythematous base with interspersed areas of aBstRaCt Incontinentia pigmenti is rare X-linked dominant disorder.There is no consistent expression of Incontinetia pigmenti in female child, but in male child, they always lead to death in utero.Vesicular, verrucous, hyperpigmented, and atrophic stages are the four stages of Incontinetia Pigmenti and it is uncommon for all stages to be seen in a same case.It is a rare genodermatosis, with only very few cases of male child with Incontinentia pigmenti have been reported.Thus, we report this case due to its extreme rarity and the child showed all the first 3 stages on followup.