Benign Cephalic Histiocytosis

Abstract

Benign cephalic histiocytosis is a self-healing non-X, nonlipid cutaneous histiocytosis of children, characterized by a papular eruption on the head. Mucous membranes and viscera are always spared. In the 13 cases reported herein, the children were otherwise in good general health. The disease appeared during the first three years of life, and spontaneous regression was complete by the age of nine years in the four cases healed to date. The histiocytic infiltrate was localized in the upper and middle dermis and contained no lipids at any stage of evolution. All the histiocytes contained coated vesicles, and 5% to 30% also contained comma-shaped bodies in their cytoplasm.

Benign Cephalic Histiocytosis with Diabetes Insipidus

“…They are usually classified either as Langerhans' cell histiocytosis (LCH) or non‐LCH, based on the pathology of the proliferating histiocytes (1). Among the main types of non‐LCH are benign cephalic histiocytosis, mononuclear (early) juvenile xanthogranuloma, xanthoma disseminatum, and generalized eruptive histiocytomas (1–13). Benign cephalic histiocytosis is rare in young children, heals spontaneously, and has not been associated with internal involvement in the 35 cases reported to date (1–13).…”

Progressive nodular histiocytomas

“…Benign cephalic histiocytosis is usually located on the face but may be generalized. However, it seems to be self‐healing 4 …”

Flat‐topped papules on the face of a young boy

“…The histiocytes stain for factor XIIIa, CD68, HAM56 (classic histiocytic marker), and demonstrate negative staining for Langerhans markers (S100).…”