Two unrelated patients with partial albinism, frequent pyogenic infections and acute
episodes of fever, neutropenia and thrombocytopenia are described. Their pigmentary
dilution was characterized by large clumps of pigments in the hair shafts and an
accumulation of melanosomes in melanocytes. Melanocytes had few short dendritic
expansions, and keratlnocytes were hypoplgmented. No or few Langerhans' cells were
detected in skin by electron microscopy and ATP-ase reactions. This pigmentary dilution …