Langerhans cell histiocytosis in children under one year

Langerhans cell histiocytosis in children under one year

Arch Argent Pediatr. 2008 Jun

Larralde M, Abad ME, Gomar B.
Hospital Alemán, Buenos Aires, Argentina.

Correspondencia: Dra. Begoña Gomar, begogomar@gmail.com

INTRODUCTION: Langerhans cell histiocytosis is characterized by a clonal proliferation of activated Langerhans cells that infiltrate various organs of the body. Occurs at any age, from newborn until adulthood, with an incidence peak at 1-4 years.

OBJECTIVE: To describe the morphologyc characteristics of skin lesions and clinical course of 15 patients with Langerhans cell histiocytosis.

METHODS: A retrospective review of the medical records of patients with Langerhans cell histiocytosis from Ramos Mejia Hospital and Aleman Hospital, between 1999-2007.

RESULTS: Review of medical records from 15 patients, 6 females and 9 males. Skin lesions were congenital in 8 cases and appeared between 2-12 months of age in 7 cases. The patients with congenital presentation only had a cutaneous manifestation; one patient who developed a systemic compromise (lung, liver and spleen) is currently under treatment. Three patients with presentation after birth only had cutaneous lesions, the others had a systemic disease. One of this patients died during treatment. Histopathology showed a histiocytic infiltrate in the papillary dermis with epidermotrophism; inmunomarking with S100 and CD1a was positive.

CONCLUSION: Both clinical manifestation (congenital and after birth) represent different ends of a spectrum of the same condition, with the potencial to develop into disseminated Langerhans cell histiocytosis.

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