Rare syndromic disorder characterized by triad of ‘yellow’ nails, peripheral oedema and pleural effusions


  • Development of yellow, thickened, and excessively curved nails
  • Complete stoppage of nail growth
  • Loss of the strip of hardened skin at the base and sides of a fingernail (cuticles)
  • Separation of the nails from the nail bed (onycholysis)
  • Infection of the soft tissue around edge of the nails (paronychia)
  • Cough and shortness of breath
  • Recurrent chest infections
  • Chronic lung condition known as bronchiectasis
  • Pleural effusions
  • Recurrent pneumonia
  • Nail changes:
  • All nails may be affected
  • Nails are slow growing or appear to have stopped growing
  • Nails become thicker and turn a pale yellow or greenish-yellow colour with edges slightly darker
  • Nails mainly remain smooth but may be cross-ridging and nail humped with loss of cuticles
  • Onycholysis (separation of nail from the nail bed) may affect one or more nails
  • Lymphoedema:
  • Swelling (most frequently affects the legs)
  • Swelling less often affects the hands, face or genitals
  • Respiratory signs:
  • Pleural effusions occur in about 36% of patients
  • In about 30% of patients, the initial symptom is related to pleural effusions
  • Patients often give a history of recurrent attack of bronchitis, chronic sinusitis, and pneumonia


  • Sporadic/acquired condition
  • Mutations of the FOXC2 gene
  • Hereditary, congenital or para-neoplastic
  • Inherited as a autosomal dominant trait

Risk factors:

  • Age: mostly seen in adults
  • Hereditary factors: increased risk in younger people

Diagnostic tests:

  • Diagnosis: based on combination of the symptoms
  • Physical observation: The nail changes
  • clinical evaluation
  • Analysis of detailed patient history
  • Identification of characteristic findings
  • Pulmonary function testing
  • Lymphogram
  • Differential diagnostics:
  • Cutaneous Melanoma
  • Irritant Contact Dermatitis
  • Lichen Planus
  • Nail Psoriasis
  • Traumatic onycholysis


  • Physical therapy like clapping or vibration
  • Postural drainage
  • Cough suppressants, sleeping pills
  • Application of liquid and cream treatments
  • Itraconazole pulse therapy
  • Diuretics and low-fat diet
  • pleurectomy
  • Pleuroperitoneal or pleurovenous shunt


  • Oral antibiotics
  • Vitamin A & E
  • Zinc sulfate
  • Itraconazole
  • Octreotide (somatostatin analogue)
  • Oral zinc supplementation

Lifestyle Management:

  • Wearing clean socks
  • Maintaining good foot hygiene
  • Rotating shoes more frequently
  • Avoiding cigarettes smoke