1 - Unidade de Cuidados Intensivos Pediátricos, Área de Pediatria, Hospital Dona Estefânia, ULS São José
2 - Departamento de Cardiologia Pediátrica, Hospital de Santa Marta, ULS S José
3 - Departamento de Pediatria, Área de Pediatria, Hospital Dona Estefânia, ULS S José 4- Serviço de Dermatologia, Hospital de Santo António dos Capuchos, ULS S José
5 - Unidade de Imunodeficiências Primárias, Área de Pediatria, Hospital Dona Estefânia, ULS S José
6 - Unidade de Gastrenterologia, Área de Pediatria, Hospital Dona Estefânia, ULS São José
7 - Unidade de Hematologia, Área de Pediatria, Hospital Dona Estefânia, ULS São José
- Divulgação: publicação Journal of Paediatrics and Chid Health 60 (2024) 876-879
Resumo:
Vascular malformations affect blood vessels: the endothelium of capillaries, arteries, veins and lymphatic vessels and their prevalence in the population ranges from 4.55% to 11.16% Multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT) is a rare and recently recognized vascular discase. It is characterized by a proliferation of multiple congenital and progressive vascular lesions usually associated with mild-to-moderate thrombocytopenia. It clinically manifests as widespread congenital reddish-brown skin papules, affects the gastro-intestinal (GI) tract with severe bleeding and can impact other organs. Thrombocytopenia, potentially linked to consumption coagulopathy is often associated with this condition. Definite diagnosis requires lesion biopsy and high morbidity, and mortality rates underscore the urgency of early recognition and targeted approaches. Treatment methods have evolved but remain challenging and controversial.
Palavras Chave: cutaneous lesions, multifocal lymphangioendotheliomatosis with thrombocytopenia