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Image Number 4804 (Lamellar ichthyosis)

 
Morphology:
Scales

Diagnosis: Lamellar ichthyosis

Site: Limbs

Sex: F

Age: 14

Type:

Description: Generalized scaling with dirty appearance.

Submitted by: Nameer Al-Sudany

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Differential Diagnosis

History:

An otherwise healthy 14 year-old girl born to nonconsanguineous marriage presented with diffuse hyper-pigmented dirty scales involving most of her body since early childhood. The girl was born in tight-cout membrane and admitted to ICU to control her general condition. Weeks later, the membrane cracked followed by slowly development of the present dirty scales. Examination revealed; multiple dirty confluent grey-black pruritic scales , of variable size involving the neck, trunk and extremities with flexural affection. Large bark-like tightly adherent scales were significantly affecting the distal extremities especially the legs. Mild degree of ectropion, bi-temporal cicatritial alopecia and palmoplantar keratoderma were noticeable. A diagnosis of lamellar ichthyosis was settled and the patient started on oral acitretin 0.5 mg/kg/day together with topical white soft paraffin in liquid paraffin (50%:50%) with proper diet rich in vitamine D.

 

This case is owned and presented by Dr. Ayman Abdlmaksoud, MD, Egypt.



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