Lysosomal Acid Lipase Deficiency: Report of Five Cases across the Age Spectrum

Figure 1

(a) Liver autopsy of case 1 showing disseminated lipid accumulation and severe steatosis with lipid-filled histiocytes (arrows) (Masson trichrome stain, 10x). (b) Duodenum biopsy of case 1 showing deformed villus, justifying the diagnosis of malabsorption syndrome. Lamina propria showing PAS+, diastasis-resistant material (arrows) (PAS+ diastase stain, 20x). (c) Liver biopsy of case 2 showing lipid-filled histiocytes and broadening portal spaces (arrows) (Masson trichrome stain, 10x). (d) Liver biopsy of case 5 showing severe macro- and microvesicular steatosis (arrows) (hematoxylin and eosin stain, 10x).

(a)

(b)

(c)

(d)