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Image Number 207 (Netherton syndrome)

 
Morphology:
Plaque

Diagnosis: Netherton syndrome

Site: Hand,dorsum

Sex: M

Age: 14

Type: Clinical

Description: Eczematous scaly plaques,Atopic eczema,ichthyosis and bamboo hair

Submitted by: Shahbaz Janjua

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Differential Diagnosis

History: This boy and his younger sister had a combination of icthyosis, eczema and alopecia since birth.  Netherton's syndrome is a rare, autosomal recessive disorder characterized by ichthyosis, hair shaft defects, and atopy.   The affected infants are usually erythrodermic with dry skin but the characteristic polycyclic and serpiginous plaques with double-edged scale(ichthyosis linearis circumflexa ) and trichorrhexis invaginata (bamboo hair) usually develop later.


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