Netherton syndrome: report of two cases

Abstract

<span>Netherton syndrome is a rare autosomal recessive condition with variable expression. It comprises an ichthyosiform dermatitis and erythroderma of variable intensity associated with hair abnormalities and features of atopy. The pathognomic (required for diagnosis) feature is trichorrhexis invaginata identified by microscopic examination of hair shaft. Ichthyosis linearis circumflexa is another typical lesion that may not always be observed. It comprises an episodic migrating erythematous and scaling annular and polycyclic flat patch with an advancing double edge of peeling scale. Here we present two cases of Netherton syndrome with characteristic manifestations mentioned above.</span>