Open AccessOlmsted syndrome: Rare occurrence in four siblings
Author(s) -
Atishay Bukharia,
Sweta Komal,
V Madhu Sudhanan,
Shyam Sundar Chaudhary
Publication year - 2016
Publication title -
indian journal of dermatology/indian journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.395
H-Index - 36
eISSN - 1998-3611
pISSN - 0019-5154
DOI - 10.4103/0019-5154.182467
Subject(s) - medicine , dermatology , acitretin , keratoderma , isotretinoin , palmoplantar keratoderma , hyperkeratosis , rare disease , lesion , deformity , pediatrics , surgery , disease , acne , psoriasis
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