Adrenal gland and paraganglia
Congenital anomalies
Storage diseases - Wolman disease

Topic Completed: 1 February 2005

Revised: 29 May 2019

Copyright: 2002-2019,, Inc.

PubMed Search: Wolman disease adrenal

See Also: Adrenoleukodystrophy  Pompe disease

Nat Pernick, M.D.
Page views in 2018: 111
Page views in 2019 to date: 202
Cite this page: Pernick N. Storage diseases - Wolman disease. website. Accessed December 11th, 2019.
Definition / general
  • Primary familial xanthomatosis
  • Rare, autosomal recessive lipid storage disorder
  • Due to deficiency of lysosomal acid lipase, causing accumulation of triglycerides and cholesterol esters in liver, spleen and adrenal glands
  • Usually causes death by age 6 months
Gross description
  • Markedly enlarged adrenal glands with dystrophic calcifications but normal architecture
Gross images

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Markedly enlarged adrenal glands

Microscopic (histologic) description
  • Necrosis, fibrosis and calcification
  • Zona fasciculata and reticularis cells have vacuolated cytoplasm
Differential diagnosis
  • Niemann-Pick disease
  • Other storage diseases
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