Presentation of lymphoedema praecox as a septic shock with acute renal failure in an adolescent girl: A case report

Abstract

Lymphoedema praecox (Meige disease) is a rare chronic disease of disordered lymphatic transport in which 10% of the cases present as non-inherited mutations responsible for defective lymphatic development. The inherent complex inflammatory pathways lead to defective lymphangiogenesis, oedema, adipose tissue deposition and chronic fibrosis. Various factors lead to local impairment of adaptive immunity leading to the increased incidence of bacterial infections. Sepsis and septic shock arising from such infection can be life-threatening. One such case is discussed where a post-pubertal adolescent girl presenting with a chronic painless unilateral limb swelling landed into cellulitis and gangrene with septic shock. She needed care in an intensive care setting and a diagnostic work-up was started to look into the cause. She recovered from this complication and care for this chronic condition was continued later. A high index of suspicion for the occurrence of this rare entity with its associated complications is the prerequisite to a successful outcome.