† Progressive cutaneous papulonodular non-X histiocytosis of the face and proximal parts of the extremities developed in a 28-year-old woman. Histologic study showed masses of benign histiocytes with foci of lymphocytes. Occasional cells contained diastase-resistant cytoplasmic polysaccharide that stained positive with PAS stain. No iron, fat, or Touton or other giant cells were present. Ultrastructural study disclosed laminar bodies but no Langerhans' cells or Langerhans' granules. The process responded to low-dose intravenous vinblastine sulfate treatment.
(Arch Dermatol 1982;118:913-917)