Ambrin Aslam, Waqar Hussain, Sajid Maqbool.
Incontinentia Pigmenti (Bloch-Sulzberger Syndrome) – A case report.
Pak Paed J Jan ;21(1):45-7.

A baby girl was born to a 22 years old healthy primigravida after a non-consanguinous marriage by spontaneous vaginal delivery at approximately 37 weeks of gestation. The birth weight was 2.25 kg. There was no history of prolonged rupture of membranes or any significant illness during pregnancy, neither was there any family history of skin disorders. The baby was born in a good condition with an Apgar score of 7 at 1 minute and 8 at 5 minutes. The systemic examination was normal but it was noticed that the skin of the limbs was covered with erythematous based vesiculo-pustular rash which was distributed in a linear pattern with hyperkeratotic pigmented lesions in between. The trunk also had pigmented lesions distributed in whorled fashion. The complete blood count showed eosinophilia of 21%. A clinical diagnosis of Incontinentia Pigmenti was made and a skin biopsy was arranged. Histopathological examination of the skin confirmed the clinically suspected diagnosis of Incontinentia Pigmenti. There were eosinophilic abcesses in the epidermis and the dermis was infiltrated with lymphocytes, polymorphonuclear leukocytes, eosinophils and mononuclear cells. The results of subsequent neurological. ophthalmic acid pediatric evaluation were normal without the detection of potential Incontinentia Pigmenti associated systemic manifestations. No specific treatment was given and the baby was discharged at five days of age.

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