Langerhans cell histiocytosis is rare and more frequent in children. The skin is
affected in 50% of the cases and is the only site in 10%. Its course varies from
self-limited and localized forms to severe multisystemic forms. Congenital cases
are usually exclusively cutaneous and self-limited, with spontaneous remission
in months. This study presents a rare congenital case, initially restricted to
the skin, with subsequent dissemination and fatal outcome. A male newborn
presented congenital disseminated erythematous scaly lesions. The biopsy was
conclusive for Langerhans cell histiocytosis. The patient evolved into the
multisystemic form in weeks, when chemotherapy was started, according to the
LCH-2009 protocol; however, the patient was refractory to treatment and
died.
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