Olmsted Syndrome: Rare Occurrence in Four Siblings.

Abstract:

:Olmsted syndrome is a very rare and severe cicatrizing keratoderma associated with periorificial lesion. Most cases are sporadic but familial occurrence has been also seen. Till now around 73 cases have been reported and none of the reported cases have 4 siblings affected from this disease. We are reporting cases of 4 siblings of age 30 year female, 26 year female, 20 year male and 10 year male who were born to a third degree consangueinous marriage and presented with palmoplantar keratoderma, periorificial hyperkeratosis, flexion deformity, pseudoainhum and contracture of digits. There was no cardiac involvement. Hence, the diagnosis of Olmsted syndrome was made and all four patients were non responsive to treatment which included topical corticosteroid, topical salicylic acid, systemic isotretinoin, systemic acitretin and oral zinc in child.

journal_name

Indian J Dermatol

authors

Bukharia A,Komal S,Sudhanan VM,Chaudhary SS

doi

10.4103/0019-5154.182467

subject

Has Abstract

pub_date

2016-05-01 00:00:00

pages

347

issue

3

eissn

0019-5154

issn

1998-3611

pii

IJD-61-347b

journal_volume

61

pub_type

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