Indian Journal of Dermatology
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E-IJD CASE REPORT
Year : 2016  |  Volume : 61  |  Issue : 3  |  Page : 347

Olmsted syndrome: Rare occurrence in four siblings


Department of Dermatology, Venereology and Leprosy, Rajendra Institute of Medical Sciences, Ranchi, Jharkhand, India

Correspondence Address:
Dr. Atishay Bukharia
Millat colony, Near Central Institute of Psychiatry (C.I.P), Kanke, Ranchi, 834006 Jharkhand
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.182467

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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.


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