Yellow-nail syndrome L60.5

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Scleronychy Syndrome; yellow nails; yellow nail syndrome

History
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Samman & White 1964

Definition
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Very rare disease characterized by the clinical triad: yellow nails, primary lymphedema and chronic respiratory diseases (mostly bronchiectasis).

Occurrence/Epidemiology
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No assured gender dominance

Etiopathogenesis
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More common in immune disorders and malignancies. Rarely also as side effect of medication ( penicillamine).

Pathogenetically, the nail changes and lymphedema are based on congenital hypoplasia of the peripheral lymph vessels.

Manifestation
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The age of onset of the disease is between 41-80 years, the mean age of onset is 60 years.

Clinical features
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Integument: Thickened nails, yellow or yellow-green in the whole nail area, slowly or not at all growing, gradually thickening nails(scleronychia) with increased cross-ribbing and peripheral onycholysis. Typical is the loss of the lunulae.

Associated extracutaneous symptoms: bronchitis (J42), bronchiectasis (J47), α-1-antitrypsin deficiency (E88.0), lymphatic vessel changes with consecutive lymphedema of the extremities (I89.0), genitals, face, vocal cords.

Laboratory
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Determination of α-1-antitrypsin.

General therapy
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Spontaneous remission possible, internal treatment of the underlying disease.

External therapy
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Note(s)
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There is evidence for the occurrence of "yellow nails" in the "lymphedema distichiasis syndrome", a clinical picture in which a mutation of the FOXC2 gene is present.

Case report(s)
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The 63-year-old patient with known silicosis of the lungs and bronchiectasis has noticed a gradual, completely painless thickening and yellowing of all fingernails for about 1 year. He hardly has to cut back his nails, which are now very difficult to cut. At the instigation of a doctor, he had carried out several local and also systemic therapies with antimycotics.

Findings: Thickened nails, yellow-greenish in the entire nail area, evenly thickened, with increased cross-ribbing. Loss of the lunulae.

Literature
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  1. Emerson PA (1966) Yellow nails, lymphoedema and pleural effusion. Chest 21: 247-253
  2. Glazer M et al (2000) Successful talc slurry pleurodesis in patients with nonmalignant pleural effusion. Chest 117: 1404-1409
  3. Venencie PY, Dicken CH (1984) Yellow nail syndrome: report of five cases. J Am Acad Dermatol 10: 187-192
  4. Samman PD, White WF (1964) The "yellow nail" syndrome. Br J Dermatol 76: 153-157
  5. Samman PD (1964) The Yellow nail syndrome: Dystrophic nails associated with lymphoedema. Trans St Johns Hosp Dermatol Soc 50: 132
  6. Scher RK et al (2003) Onychomycosis in clinical practice: factors contributing to recurrence. Br J Dermatol 149(Suppl 65): 5-9
  7. Tosti A et al (2002) Systemic itraconazole in the yellow nail syndrome. Br J Dermatol 146: 1064-1067
  8. Valdés L et al (2014) Characteristics of patients withyellow
    nail syndrome and pleural effusion. Respirology 19:985-992

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Last updated on: 29.10.2020