Langerhans Cell Histiocytosis as Cause of Central Diabetes Insipidus: Case Report

Abstract

Langerhans cell histiocytosis (LCH) is a rare disease of the monocyte-macrophage system, characterized by the aberrant proliferation of specific dendritic cells. The clinical presentation ranges from a single bone lesion to widespread multiorgan involvement. This disease is usually considered to be a disease of childhood; however, the diagnosis is frequently made in adulthood. The course of the disease is fairly unpredictable and varies from spontaneous resolution to progress into a debilitating form, which compromises the vital functions with occasional fatal consequences. Langerhans cell histiocytosis exhibits a predilection for the hypothalamic-pituitary-axis, with diabetes insipidus being the most common endocrine consequence related to the disease, which may be prior to diagnosis or develop at any time during the course of the disease. The diagnosis of LCH should be based on histologic and immunophenotypic examination of a lesional biopsy, although other testing may be done, depending on the symptoms. There is no established, widely agreed-upon treatment of LCH, in general. The treatment depends upon the individual patient and the extent and areas of involvement. The present article aims to describe the case of a 26-year-old male patient whose symptoms started with a headache and occipital bone lesion that progressed later with diabetes insipidus.

Resumo

A histiocitose de células de Langerhans (HCL) é uma rara doença do sistema monocítico-macrofágico, caracterizada pela proliferação aberrante de células dendríticas específicas. As manifestações clínicas variam de uma única lesão óssea a um acometimento extenso de múltiplos órgãos. Esta doença geralmente é considerada uma doença da infância; no entanto, o diagnóstico é frequentemente feito na idade adulta. O curso da doença é bastante imprevisível e varia de resolução espontânea a progressão para uma forma debilitante, com comprometimento de funções vitais e ocasionais consequências fatais. A HCL exibe uma predileção pelo eixo hipotálamo-hipofisário, sendo o diabetes insípido a consequência endócrina mais comum relacionada à doença, podendo ser anterior ao diagnóstico ou se desenvolver a qualquer momento durante o curso da doença. O diagnóstico de HCL deve basear-se nos exames histológico e imunofenotípico de uma biópsia lesional, embora outros exames possam ser feitos a depender dos sintomas apresentados pelo paciente. Não existe um tratamento estabelecido, amplamente aceito para a HCL, em geral. O tratamento depende individualmente do paciente, da extensão e das áreas de envolvimento. O presente artigo tem como objetivo descrever o caso de um paciente do sexo masculino de 26 anos de idade que iniciou o quadro com uma cefaleia e lesão óssea occipital que evoluiu posteriormente com quadro de diabetes insípido.

Keywords

histiocytosis - Langerhans cell - pituitary gland - hypothalamo-hypophyseal system - diabetes insipidus

Palavras-chave

histiocitose de células de Langerhans - hipófise - sistema hipotálamo-hipofisário - diabetes insípido

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