Netherton’s Syndrome: A Case Report

Nidal A. Obaidat, Faten Al-Awaysheh, Rahmeh Fayez

Abstract


We report a case of a 12.5 years old male patient who presented to the dermatology clinic complaining of pruritic scaly dry skin and sparse brittle hair. Clinical and laboratory assessment revealed ichthyosis linearis circumflexa, trichorrhexis and atopy. These findings fit the diagnosis of Netherton′s syndrome. Family history is also positive for the same disease.
Netherton′s Syndrome (NS) is a rare autosomal recessive disorder, first described by Netherton in 1958 in a girl with erythematous scaly dermatitis who had 'bamboo-like nodes' in her sparse fragile hairs. 1 The classical triad of clinical features includes ichthyosis, hair shaft abnormalities and atopic diathesis. 2, 3 The disease has an estimated incidence of 1 in 100,000. 2 Only three cases have been published from the Arab World, 4, 5, 6 and to the best of our knowledge, our case is the first to be published from Jordan.

Keywords


Netherton′s syndrome, case report.

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