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Vitiligo and Pernicious Anemia

Fritz Egli, M.D., and Roland Walter, M.D.

N Engl J Med 2004; 350:2698June 24, 2004

Article

A 45-year-old woman with a 17-year history of vitiligo (panel A) was evaluated because of persistent fatigue. She was found to have pancytopenia (white-cell count, 3200 per cubic millimeter; hemoglobin level, 8.1 g per deciliter; and platelet count, 147,000 per cubic millimeter) with macrocytosis (mean corpuscular volume, 125 μm3) and hypersegmented polymorphonuclear leukocytes (Panel B; hematoxylin and eosin, ×100). The serum vitamin B12 level was 84 pmol per liter (normal range, 220 to 660). The serum folate level was normal. Erythroid hyperplasia with left-shifted megaloblastic erythropoiesis and giant metamyelocytes (Panel C, arrow; hematoxylin and eosin, ×100) was seen in the marrow aspirate. Gastric biopsy (specimen not shown) revealed severe alterations of the mucosa with glandular atrophy and intestinal metaplasia. The diagnosis of chronic atrophic gastritis type A (autoimmune) was supported by the finding of markedly elevated serum levels of antibodies to gastric parietal cells (1:260; normal value, <1:10). The patient was treated with intramuscular vitamin B12, and the hematologic abnormalities completely resolved.

Increasing evidence suggests that vitiligo is an autoimmune disorder. It may be associated with other autoimmune diseases, such as pernicious anemia, rheumatoid arthritis, type 1 diabetes, alopecia areata, and diseases of the thyroid gland, or it may be a manifestation of a polyglandular autoimmune syndrome.

Fritz Egli, M.D.
Roland Walter, M.D.
Kantonsspital, CH-7000 Chur, Switzerland