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   Introduction
   Case Report
   Discussion
   References
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CASE REPORT
Year : 1998  |  Volume : 64  |  Issue : 5  |  Page : 235-236

Siemens syndrome



Correspondence Address:
Laxman Mavarkar


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Source of Support: None, Conflict of Interest: None


PMID: 20921778

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How to cite this article:
Mavarkar L. Siemens syndrome. Indian J Dermatol Venereol Leprol 1998;64:235-6

How to cite this URL:
Mavarkar L. Siemens syndrome. Indian J Dermatol Venereol Leprol [serial online] 1998 [cited 2019 Jun 15];64:235-6. Available from: http://www.ijdvl.com/text.asp?1998/64/5/235/4706





  Introduction Top


A large group of localised keratodermas has been distinguished principally on morphological grounds, comprising areata, striata and punctate keratodermas. There are demonstrable genetic differences between many of these disorders but differentiation based on morphology alone has led to voluminous and unrewarding leterature.[1] Keratosis palmoplantaris striata is known as Siemens syndrome.[2] Herewith we report a case of Siemens, syndrome for its rarity.


  Case Report Top


A 10-year-old girl born of consanguinous marriage present with raised skin lesion over planatar aspect of the feet since early childhood. There was history of physical and mental ratardation and deafness since infancy. There was no involvement of hair or teeth. And also there was no history of similar illness in the family.

General and systemic examination revealed mental as well as physical retardation [Figure - 1]. Cutaneous examination revealed welll-defined islands of multiple hyperkeratotic lesions over the soles [Figure - 2]. The palms were normal. Hair, teeth, and nails were also normal.

Routine blood and urine investigations were within normal limits. Serum calcium, phosphorous, TSH, T3 and T4 levels were also within normal limits. Skeletal survey showed only minimal osteoporosis. Audiogram showed bilateral mild conductive hearing loss. Based on the clinical and laboratory findings, the patient was diagnosed as Siemens syndrome.


  Discussion Top


Siemens syndrome is inherited as an autosomal domiant. The earliest sign is palmar or plantar erythema followed by islands or linear hyperkeratotic lesions.[3] Associated features commonly encountered are teeth enamel dyspiasia, total leuconychia, steely hair and deafness.[4] But in our case deafness was associated with physical as well as mental retardation.



 
  References Top

1.Griffiths WAD, Leigh IM, Marks R. Disorder of keratinisation. In Texbook of Dermatology Champion RH, BurtenJI, Ebling FJG, eds, 5th Edition, Oxford: Blackwell Scientific Publications, 1992;1325-1390.  Back to cited text no. 1    
2.Siemens H W. Keratosis palmoplantaris striata. Arch Dermatol Syphilol 1929;157:392-408.  Back to cited text no. 2    
3.Kientz T. Keratosis palmoplantris striata, Brunnausr Fuhs 1927 (Keratosis palmoplantaris various wachtterss 1963). Hautarzt 1976;27:403.  Back to cited text no. 3    
4.Crosti C, Sala F, Leuconychic totale et dyspiasia ectodermique; observation de deur cas. Ann Dermatol Venereol 1983;110:617-622.  Back to cited text no. 4    


    Figures

[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]



 

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Online since 15th March '04
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