Benign cephalic histiocytosis: report of four cases.

We cared for four patients with benign cephalic histiocytosis, a self-healing non-X, nonlipid cutaneous histiocytosis of children. The age of onset of the disease was 5 to 9 months, with papules and erythematous macules involving the head (mainly the cheeks), and posterior spread to the trunk and limbs in three patients. Microscopic examination of skin biopsies revealed a histiocytic infiltrate in the superficial dermis that was S100 protein-negative by immunoperoxidase (PAP method). One patient showed comma-shaped bodies and desmosomelike junctions on electron microscopy. No Birbeck's granules were present. Benign cephalic histiocytosis is a self-limiting condition that requires no treatment.