Griscelli syndrome.

A 4-month-old child had silvery gray hair, light-colored skin, recurrent chest infections, hepatosplenomegaly, and episodes of pancytopenia and hemophagocytosis in the liver, spleen, and bone marrow. Light microscopy of hair showed characteristic large aggregates of pigment granules distributed irregularly along the hair shaft. Peripheral blood smear examination did not show giant granules in granulocytes. Enlarged hyperpigmented basal melanocytes with sparsely pigmented adjacent keratinocytes were seen on the skin biopsy specimen. On the basis of these clinical and laboratory findings, Griscelli syndrome was diagnosed. The child succumbed to infection during an accelerated phase of the disease.

• Publication year

  2020

• Venue

  Journal of American Academy of Dermatology

• Publication date

  2020-02-10

• Fields of study

  Medicine

• Identifiers
  DOI 10.1016/j.jaad.2005.11.1056PMID 16844525
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

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