Congenital self-healing langerhans cell histiocytosis: clinical and pathological characteristics.

Introduction: Congenital Self-Healing Langerhans Cell Histiocytosis (CSHLCH) is rare, characterized by cutaneous lesions at birth or in the neonatal period, and absence of systemic lesions.

Materials And Methods: Skin biopsy was performed and the histologic examination of the skin section, routinely stained with hematoxylin-eosin. Paraffin sections were stained by immunohistochemical study which were carried out according to the manufacturer's protocols.

Result: Seven cases of CSHLCH were recruited. 28.6% (2/7) of the cutaneous lesions were multiple, 71.4% (5/7) were solitary. Skin biopsy was performed and the histologic examination of the skin section, routinely stained with hematoxylin-eosin. Microscopically, in the dermis, a dense infiltrate of histiocytic cells mixed with numerous eosinophils. These histiocytes were immunohistochemically positive for CD1a and S-100. All of the cutaneous lesions regress spontaneously, and lack of systemic involvement, the final diagnosis of Congenital Self-Healing Langerhans Cell Histiocytosis were made. No recurrence had been observed.

Conclusion: The cutaneous lesions of CSHLCH may regress spontaneously. Spontaneous resolution of cutaneous lesions and lack of systemic involvement are essential for the diagnosis of CSHLCH. It needs long-term follow-up.