|Year : 1993 | Volume
| Issue : 1 | Page : 15-16
Linear scleroderma in a family
Vineet Kaur, Gurpreet Singh
Source of Support: None, Conflict of Interest: None
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
| Introduction|| |
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|| |
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|| |
Scleroderma in more than one member of a family is very rare. Reports of such cases are only few.
Rees and Burnett  reported localized scleroderma in a father and daughter. Christianson et al  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  found 13 familial instances of scleroderma occurring in diverse forms and combinations. Wuthrich et al  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|| |
|1.||Rees RB,Bennett J. Localized Scleroderma in father and daughter. Arch Dermatol 1953; 68: 360. [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. |
|3.||Burge KM, Perry HO, Stickler GB. Familial scleroderma. Arch Dermatol 1969; 99: 681-7. [PUBMED] |
|4.||Wuthrich RC, Henry H, Roenigk Jr, Willard D S. Localized scleroderma. Arch Dermatol 1975; 111: 98-100. |
[Figure - 1], [Figure - 2]