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Indian Pediatr 2010;47: 1052 |
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Ichthyosis Linearis Circumflexa |
Piyush Kumar, RC Gharami, Avijit Mondal and Kalyan Ghosh,
Department of Dermatology, Medical College, Kolkata,
India.
Email: [email protected]
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A 7 years old girl, born of a non-consanguineous marriage, presented with
multiple asymptomatic dry and scaly patches over her body since infancy.
The patches were constantly changing their shape and pattern, resolving in
2-3 weeks with mild transient hypopigmentation, and were recurrent.
Lesions tended to get better in summer with aggravation in winter. On
examination, she had multiple, erythematous annular and polycyclic scaly
patches with double-edged scales at the periphery of the lesions. They
were present bilaterally and in asymmetric manner over face, neck, trunk
and extremities. Hair, nail and mucosa were normal (Fig. 1).
Clinically the child was diagnosed as Ichthyosis linearis circumflexa (ILC).
Routine investigations, KOH mount and Wood’s lamp examination were
unremarkable. Histopatho-logical findings were consistent with this
diagnosis. The child was treated with oral isotretinoin 10 mg a day for 1
month and lesions resolved within 2 weeks. No new lesions have appeared
since then. ILC is a rare autosomal recessive disorder of keratinisation.
Association of ILC, hair abnormality and atopic diathesis is called
Netherton’s syndrome (NS). Common differential diagnoses include
erythrodermic atopic/ seborrhoeic eczema, staphylococcal/ Candida
infection, tinea corporis and familial peeling skin syndrome. In neonatal
period, intensive medical, nursing and nutritional care must be available
to manage erythroderma and its complications. In older children, regular
emollients (e.g. ammonium lactate 12%) are helpful.
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Fig. 1 Lesions distributed symmetrically on
trunk. |
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