Griscelli syndrome - 09/08/11
Correspondence to: M. Ramam, MD, Department of Dermatology and Venereology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110029, India.New Delhi, India
Abstract |
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.
Le texte complet de cet article est disponible en PDF.Abbreviations used : CHS, ES, GS
Plan
| Funding sources: None. Conflicts of interest: None identified. Presented at the 33rd National Conference of Indian Association of Dermatologists, Venereologists and Leprologists, New Delhi, India, February 3-6, 2005. Reprints not available from the authors. |
Vol 55 - N° 2
P. 337-340 - août 2006 Retour au numéro
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