ICD-11 classes
14 Diseases of the skin
Skin disorders involving specific cutaneous structures
Disorders of the epidermis and epidermal appendages
Disorders of the nail or perionychium
EE11 — Acquired abnormalities of nail colour
EE11.1 — Yellow nail syndrome

ICD-11 EE11.1 — Yellow nail syndrome

Yellow nail syndrome is characterised by yellow, dystrophic, thick and slowly growing nails, associated with lymphoedema and respiratory involvement. Less than 100 cases have been described. Lymphoedema occurs more often in the lower limbs. It can appear at birth or later in life. Onset generally follows the onset of ungual abnormalities. Patients usually suffer from chronic bronchitis and in some cases, from chronic sinusitis, bronchiectasia and recurring pneumonitis. They can also present with pleural effusion (30% of cases) and bronchial hyperreactivity. Most cases are sporadic. However, familial forms have been described. Aetiology is unknown.

The diagnosis includes nothing.

The diagnosis excludes nothing.

It has no clarifying diagnoses.

The diagnosis is coded elsewhere:

• Primary lymphoedema #11642
• Primary lymphoedema #11664
• Primary lymphoedema #12174
• Pulmonary lymphatic dysplasia syndromes #12745
• Primary lymphoedema #16675
• Primary lymphoedema #16697
• Cutaneous markers of internal disorders #17427
• Primary lymphoedema #22708
• Primary lymphoedema #23223