Larralde Margarita, Abad María E, Gomar Begoña
Hospital Alemán, Buenos Aires, Argentina.
Arch Argent Pediatr. 2008 Jun;106(3):269-72. doi: 10.1590/S0325-00752008000300015.
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.
To describe the morphologyc characteristics of skin lesions and clinical course of 15 patients with Langerhans cell histiocytosis.
A retrospective review of the medical records of patients with Langerhans cell histiocytosis from Ramos Mejia Hospital and Aleman Hospital, between 1999-2007.
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.
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.
