Very rare. Most cases are sporadic, but familial forms have been described.
Syndrome characterized by the presence of yellow, dystrophic, thick and slowly growing yellow nails, associated to lymphoedema and respiratory impairment. The pathogenesis is not known: the fact that oedema is reversible suggests that lymphatic involvement is secondary or functional, even if hypoplasia or absence of lymphatic vessels is sometimes present in the area affected by lymphedema. The diagnosis is not easy because the symptoms do not necessarily appear at the same time.
Clinical signs:
- yellow nails: yellow to green, dystrophic, longitudinally and transversely curved nails, with absence of lunula and cuticle; distal onycholysis is sometimes presnet. Nail growth is very slow. Fungal cultures are negative.
- lymphedema: most frequently affecting the lower limbs. It can be present at birth or appear later. Unlike conventional forms, this lymphedema can regress.
- respiratory involvement: chronic bronchitis and sometimes chronic sinusitis, bronchiectases and recurrent pneumonia, pleural effusion (30-40% of cases, with a chylothorax in 30% of cases) and bronchial hyperresponsiveness. In children, history of hydrops fetalis is frequently positive.
Some cases of exudative enteropathy have been reported with yellow nail syndrome. The local application of vitamin E and itraconazole by mouth may improve the aspect of the nails.
Anesthetic implications:
evaluate the respiratory involvement; chest XRays (pleural effusion, bronchectasies), respiratory evaluation, exclude chronic infectious spots
References :
- Yalçin E, Dogru D, Gönç EN, Cetinkaya A, Kiper N.
Yellow nail syndrome in an infant presenting with lymphedema of the eyelids and pleural effusion.
Clin Pediatr 2004;43:569–72.
- Dessart P, Deries X, Guérin-Moreau M, Troussier F, Martin L.
Syndrome des ongles jaunes : deux cas pédiatriques.
Ann Dermatol Vénéréol 2014 ; 141 :611-9
- Maldonado F, Ryu JH.
Yellow nail syndrome.
Curr Opin Pulm Med 2009 ; 15 : 371-6.
- Valdes L, Huggins JT, Gude F, Ferreiro L et al.
Characteristics of patients with yellow nail syndrome and pleural effusion.
Respirology 2014 ; 19 : 985-92.
- Aslam MZ, De Loughry G, O’Brien B.
Anaesthesia and orphan diseases: yellow nail syndrome.
Eur J Anaesthesiol 2020; 137: 728-30.
Updated: July 2020