Indian Journal of Dermatology
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Year : 2013  |  Volume : 58  |  Issue : 2  |  Page : 165
A child in horse-riding stance


Department of Dermatology, Venereology and Leprosy, Sri. B. M. Patil Medical College, Hospital and Research Center, BLDE University, Bijapur, Karnataka, India

Date of Web Publication5-Mar-2013

Correspondence Address:
Arun C Inamadar
Department of Dermatology, Venereology and Leprosy, Sri. B. M. Patil Medical College, Hospital and Research Center, BLDE University, Bijapur, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.108120

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How to cite this article:
Inamadar AC, Palit A, Ragunatha S. A child in horse-riding stance. Indian J Dermatol 2013;58:165

How to cite this URL:
Inamadar AC, Palit A, Ragunatha S. A child in horse-riding stance. Indian J Dermatol [serial online] 2013 [cited 2019 Oct 18];58:165. Available from: http://www.e-ijd.org/text.asp?2013/58/2/165/108120


A 6-year-old girl presented with progressive tightness of skin over pelvic and shoulder girdles resulting in stiffness of extremities giving her a fixed horse-riding stance [Figure 1]. It started as an ill-defined stony hard area noticed on the lateral side of right buttock at 2 years of age and there was a gradual progression of skin induration over proximal extremities. There was exaggerated lumbar lordosis, outer aspects of both the buttocks were flat and hollowed, and lower back and lumbar region showed mild hypertrichosis [Figure 1] and [Figure 2]. Skin on the involved body parts was rock-hard and not pinchable. Face, neck, hands, and feet were not involved. There was no history of Raynaud's phenomenon, arthralgia, dyspnea, or dysphagia. Hemogram, ESR, random blood sugar, renal function, and urinalysis were within normal limits. Antinuclear antibody test was negative. A deep biopsy was taken from an involved area of her left thigh including skin and underlying fascia.
Figure 1: Horse-riding stance of the child

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Figure 2: Hollowed contour of the buttocks with mild hypertrichosis of the lumbo-sacral region

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   Histopathological findings Top


There were (H and E) unremarkable epidermis, sparse, perivascular lymphocytes in the upper dermis and thick, sclerotic collagen bundles in the reticular dermis. There was no evidence of appendageal distortion. Subcutaneous fat and fascia were normal in appearance in the biopsied specimen.


   Question Top


What is your Diagnosis?



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   References Top

1.Liu T, McCalmont TH, Frieden IJ, Williams ML, Connolly MK, Gilliam AE. The stiff skin syndrome. Case series, differential diagnosis of the stiff skin phenotype and review of the literature. Arch Dermatol 2008;144:1351-9.  Back to cited text no. 1
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2.Jablonska S, Groniowski J, Krieg T, Nerlich A, Peltonen L, Oikaryndinen A, et al. Congenital fascial dystrophy: A noninflammatory disease of fascia: The stiff skin syndrome. Pediatr Dermatol 1984;2:87-97.  Back to cited text no. 2
    
3.Caspary P, Costa F, Martins Souza PR. Familial localized stiff skin syndrome. Int J Dermatol 2010;49:842-8.  Back to cited text no. 3
    
4.Loeys BL, Gerber EE, Riegert-Johnson D, Iqbal S, Whiteman P, McConnell V, et al. Mutations in fibrillin-1 cause congenital scleroderma: Stiff skin syndrome. Sci Transl Med 2010;2:23ra20.  Back to cited text no. 4
[PUBMED]    
5.Fidzianska A, Jablonska S. Congenital fascial dystrophy: Abnormal composition of the fascia. J Am Acad Dermatol 2000;43:797-802.  Back to cited text no. 5
[PUBMED]    
6.Pages ON, Maliszewicz P, Lefebvrel F, Poli-Merol ML, Morville P. Visceral involvement in stiff skin syndrome. Pediatr Dermatol 2010;27:327.  Back to cited text no. 6
    


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