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Incontinentia pigmenti (Bloch-Sulzberger syndrome) is an uncommon genodermatosis mainly affecting females. It consists of characteristic skin manifestations associated in 79.8% of patients with other systemic manifestations. Of these there have been five cases of palatal or lip abnormalities, none being bilateral cleft lip and palate. We describe a unique case of incontinentia pigmenti associated with a bilateral cleft lip and palate.
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Yell et al. (1991) studied this question.
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