Incontinence pigmenti (Bloch-Sulzberger syndrome) in the neonatal stage A case report.

Abstract

Introduction: Incontinence Pigmenti (IP) is a rare neurocutaneous disorder caused by the hereditary alteration linked to the X chromosome; a clinical case is presented.

Case report: A newborn girl who is evaluated at 48 hours of age for skin lesions with erythematous vesicles-pustular blisters located on the left lower limb, present from birth. The lesions spread, increasing in number to other regions and sustaining irritability.

Diagnostic workshop: A skin biopsy was performed, and incontinence pigmenti was diagnosed. The genetic study determined a dominant genodermatosis linked to the X chromosome, which is associated with the IKBKG GENE mutation. Hematological, chemical, and hormonal control studies were regular. There was no neurological or ophthalmic involvement.

Evolution: The patient, with lesions in the vesicular phase, received topical treatment with zinc oxide and calamine. During its evolution, infected papule-type lesions were added, which is why oxacillin was required. She was discharged in better condition and remains under observation.

Conclusions: In the present case, differential diagnoses such as bullous impetigo, bullous pemphigoid, neonatal herpes, cytomegalovirus, mastocytosis, hereditary epidermolysis bullosa, or toxic erythema are proposed.