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Primary spontaneous cerebrospinal fluid leaks occurring in the absence of any apparent cause are rare. Patients may present such leaks simultaneously, or successively, in multiple locations. We report here the sixth case of anterior and lateral skull base defects presented in a single patient. Although rhinoliquorrhea and otoliquorrhea were reported separately in the literature, we postulated that the pathophysiology is the same for the whole skull base; obesity seems to contribute to the development of such primary spontaneous cerebrospinal fluid leak. Consequently, management is similar and regular follow-up is very important.

Lipoid proteinosis is a rare autosomal recessive condition affecting the majority of organ systems, but predominantly involving skin and mucous membranes. The mucocutaneous infiltration due to accumulation of a hyaline material is positive for both sudanophil and periodic acid-Schiff reagents. This was illustrated in a 14-year-old girl with parental consanguinity, who had classic manifestations.