Incontinentia pigmenti associated to cleft palate. Case report and literature review

Maria Antonieta Inostroza H, Francisco Javier Verdugo A


Incontinentia pigmenti (IP2, Block-Sulzberger Syndrome) is a rare x-linked dominant genetic skin disease mainly affecting females. Its manifestations, among many others, consist of a series of skin, dental ocular  and neurological disorders.

Patient and Method

14 month old female patient . At birth  vesicular lesions were observed in legs, underarms ( armpits) and buttocks area. Upon breaking, the lesions developed into  erythematosus and desquamative lesions. Other observed signs were vertex alopecia and cleft palate.


The clinical geneticist confirmed the presence of X-linked dominant syndrome, since the mother presented the same lesions at birth. The patient was referred to the maxillofacial service for cleft palate treatment.


Skin alterations present at  birth might precede others in the dental area, they thus warrant  preparation  for further prevention and treatment phases.

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