Netherton Syndrome - Key Clinical Features

(1) Congenital exfoliative erythroderma

(2) Cutaneous redness and peeling

(3) Hypernatremic dehydration in the neonatal period

(4) Ichthyosis linearis circumflexa (migratory serpiginious scaling erythema with peripheral double edged scale)

(5) Thin, sparse, fragile, slow growing hair

(6) Trichorrhexis invaginata (bamboo hair) on light microscopy examination

(7) Sparse eyelashes and eyebrows

(8) Allergic manifestations

(9) Atopic dermatitis with elevated serum lgE concentrations

(10) Chronic and severe skin inflammation

(11) Pruritus

(12) Asthma

(13) Hay fever

(14) Multiple food allergies

(15) Lichenification of the flexures

(16) Recurrent infections

(17) Failure to thrive

(18) Malnutrition