Lipoid proteinosis: case report

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.