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Congenital self-healing langerhans cell histiocytosis with atrophic recovery of the skin: clinical correlation of an immunologic phenomenon.

The pathophysiology and pathogenesis of congenital self-healing Langerhans cell histiocytosis (CSHLCH) as well as that of the other types of Langerhans cell histiocytosis is not well understood. Some authors postulate deregulated immunologic mechanisms that result in overproduction of cytokines. We examined a neonate with disseminated papulonodular eruptions containing lymphoid aggregates of B lymphocytes in the chorial layer. The diagnosis of a CSHLCH was made and the follow-up showed a complete involution of the eruptions, leaving atrophic lesions in the sites corresponding to the initial findings. We discuss a possible imbalance of the immune response as a pathogenetic mechanism.