Histiocitose de células de Langerhans: relato de caso e revisão da literatura

Abstract

Os autores descrevem caso de paciente de dois anos de idade, apresentando lesões pápulovesiculosas no tronco que evoluíram para crostas, além de tumorações com necrose na região cervical. Durante a internação, observou-se estrabismo convergente à direita, de instalação súbita. Os exames histopatológico e imuno-histoquímico das lesões cutâneas comprovaram tratar-se de histiocitose de células de Langerhans, sendo o paciente submetido à quimioterapia com melhora importante do quadro.

Histiocitose de células de Langerhans com acometimento vulvar e com resposta terapêutica à talidomida: relato de caso

“…7 Extensive cutaneous lesions can be treated with low dose systemic corticotherapy and chemotherapy (usually a sole drug, most commonly vinblastine, methotrexate and etoposide). 1,7,9 More recently some studies have shown a good response to thalidomide. 6,11 The drug has antinflammatory and antineoplastic effects and works through inhibition of TNF and IL-6, which have increased expression in LCH.…”

“…1 The ethiology is unknown and some believe it is probably a secondary condition to viral infections or immunologic imbalance; neoplasic and genetic origins have also been proposed. 1,4,6,7,9 It can develop at any age but the incidence peak is between 1 and 3 years of age. 2 Patients with focal lesions are usually older than those with multisystem disease.…”

“…1,3,10 The ideal therapy for LCH has not yet been established, and varies between cytotoxic or immunomodulatory drugs. 9 The clinical variant of the disease and the fact that 10 to 20% of the patients have spontaneous regression make it difficult to compare between different therapies and the majority of the available options are based on small case reports.…”

“…1,7 Local radiotherapy is low doses can be associated with chemotherapy in cases with more extensive bone involvement. 7 Prednisone is the drug of choice in case of lung involvement.…”

“…11 The prognosis of LCH is worse in those under two years of age and the forms with exclusively cutaneous and/or bone lesions tend to have a better outcome. 1,2 Morbidity is related to the structural distortions related to the disease, like pulmonary fibrosis, hepatic cirrhosis, glandular and cognitive dysfunctions. 1,5 Follow-up must be indefinite since the course of the disease after remission is unpredictable, and systemic or malignant disease might develop concomitantly.…”