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