Olmsted syndrome: Rare occurrence in four siblings
Autor: | V Madhu Sudhanan, Atishay Bukharia, Shyam Sundar Chaudhary, Sweta Komal |
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Jazyk: | angličtina |
Rok vydání: | 2016 |
Předmět: |
medicine.medical_specialty
Hyperkeratosis Dermatology Disease Acitretin Lesion 030207 dermatology & venereal diseases 03 medical and health sciences 0302 clinical medicine medicine lcsh:Dermatology Olmsted syndrome Keratoderma Isotretinoin E-IJD Case Report business.industry periorificial palmoplantar keratoderma lcsh:RL1-803 medicine.disease Surgery Palmoplantar keratoderma Consanguineous marriage 030220 oncology & carcinogenesis Contracture medicine.symptom business medicine.drug |
Zdroj: | Indian Journal of Dermatology, Vol 61, Iss 3, Pp 347-347 (2016) Indian Journal of Dermatology |
ISSN: | 1998-3611 0019-5154 |
Popis: | 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. |
Databáze: | OpenAIRE |
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