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Paper Information

Journal:   IRANIAN JOURNAL OF DERMATOLOGY   FALL 2009 , Volume 12 , Number 3 (49 SUPPLEMENT); Page(s) 51 To 54.
 
Paper: 

KINDLER SYNDROME: 2 CASE REPORTS FROM INDIA

 
 
Author(s):  SALODKAR A.*, CHOUDHARY S., KOLEY S.
 
* JAWAHARAL NEHRU MEDICAL COLLEGE SAWANGI, MAHARAASHTRA STATE, INDIA
 
Abstract: 

Kindler syndrome (KS) is a rare autosomal recessive genodermatosis. We report two cases of KS with classical clinical presentations involving skin and mucus membranes. Clinically, both patients had four major features of KS in the form of acral skin blistering, photosensitivity, progressive poikiloderma, and diffuse cutaneous atrophy. Case 1 had associated features in the form of urethral stenosis, skin fragility and palmoplantar keratoderma with extension of the scaling onto the flexor aspect of the wrist and loss of palmar creases. Case 2 had associated features in form of anal stenosis, oesophageal stenosis, skin fragility and palmoplantar keratoderma with loss of palmar creases. An Interesting finding in our report is that both cases have prominent telengectasia involving face and neck regions.

 
Keyword(s): ACRAL SKIN BLISTERING, PHOTOSENSITIVITY, POIKILODERMA, KINDLER SYNDROME, CUTANEOUS, ATROPHY
 
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