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CASE REPORT
Year : 1993  |  Volume : 59  |  Issue : 1  |  Page : 15-16

Linear scleroderma in a family




Correspondence Address:
Vineet Kaur


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Source of Support: None, Conflict of Interest: None


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  Abstract 

A rare occurrence of localized scleroderma in a mother and daughter is reported. This is perhaps the first reported family in Indian literature.


Keywords: Scleroderma, Morphoea


How to cite this article:
Kaur V, Singh G. Linear scleroderma in a family. Indian J Dermatol Venereol Leprol 1993;59:15-6

How to cite this URL:
Kaur V, Singh G. Linear scleroderma in a family. Indian J Dermatol Venereol Leprol [serial online] 1993 [cited 2019 Sep 16];59:15-6. Available from: http://www.ijdvl.com/text.asp?1993/59/1/15/3868



  Introduction Top


Localized scleroderma is a disorder of unknown aetiology. It's familial occurrence is extremely rare. We report a family in which mother had scleroderma en coup-de-sabre and daughter had linear scleroderma.


  Case Reports Top


Mother : A 35-year old woman noticed slight hyperpigmentation on the right side of the face since the age of 15 years. Later skin over the affected area became firm. A linear groove appeared on the sides of the forehead extending into the scalp. Hair over this area were gradually lost [Figure - 1]. These changes did not progress after the initial 5 years.

Examination revealed bound down skin over the right cheek including alae nasi and the frontoparietal region. There was a linear band of alopecia. No other abnormality was found.

Daughter : A 13-year-old girl presented with an area of dried up and shiny skin over her right leg and thigh for the last 3 years.

On examination, skin over the right shin and thigh was hyperpigmented and bound down. Hair over the affected area were lost. There were areas of patchy hyperkeratosis [Figure - 2]. No other abnormality was detected. Histopathological examination of the affected skin in both subjects confirmed the diagnosis of scleroderma.


  Comments Top


Scleroderma in more than one member of a family is very rare. Reports of such cases are only few.

Rees and Burnett [1] reported localized scleroderma in a father and daughter. Christianson et al [2] studied 235 patients of localized scleroderma and found only 2 instances in which 2 family members were involved. In a review of literature, Burge et al [3] found 13 familial instances of scleroderma occurring in diverse forms and combinations. Wuthrich et al [4] added 2 more families to literature, one having 3 children and the other 2 with localized scleroderma.

The present family is perhaps the first being reported in Indian literature. Localized scleroderma in one member is of en-coup-de sabre type and in the other is of linear type. These reports suggest that there is some genetic predisposition to develop sclerodermatous process.

 
  References Top

1.Rees RB,Bennett J. Localized Scleroderma in father and daughter. Arch Dermatol 1953; 68: 360.  Back to cited text no. 1  [PUBMED]  
2.Christianson HB, Dorsey CS, O'Leary PA, et al. Localized scleroderma: a clinical study of 250 cases. Arch Dermatol 1956; 74: 629-39.  Back to cited text no. 2    
3.Burge KM, Perry HO, Stickler GB. Familial scleroderma. Arch Dermatol 1969; 99: 681-7.  Back to cited text no. 3  [PUBMED]  
4.Wuthrich RC, Henry H, Roenigk Jr, Willard D S. Localized scleroderma. Arch Dermatol 1975; 111: 98-100.  Back to cited text no. 4    


    Figures

[Figure - 1], [Figure - 2]



 

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