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   Introduction
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CASE REPORT
Year : 1992  |  Volume : 58  |  Issue : 4  |  Page : 280-282

Ehlers - Danlos syndrome type II (mitis)




Correspondence Address:
S N Tolat


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Source of Support: None, Conflict of Interest: None


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  Abstract 

A case of a mild subvariant of Ehlers-Danlos Syndrome Type II (mitis) is reported. A clinical scoring system to diagnose such cases in highlighted.


Keywords: Ehlers-Danlos syndrome


How to cite this article:
Tolat S N, Gokhale R K. Ehlers - Danlos syndrome type II (mitis). Indian J Dermatol Venereol Leprol 1992;58:280-2

How to cite this URL:
Tolat S N, Gokhale R K. Ehlers - Danlos syndrome type II (mitis). Indian J Dermatol Venereol Leprol [serial online] 1992 [cited 2019 Oct 16];58:280-2. Available from: http://www.ijdvl.com/text.asp?1992/58/4/280/3819



  Introduction Top


In 1682 van Meekeren described the first definitive case of  Ehlers-Danlos syndrome More Details (EDS). Ehlers recognized the loose jointedness and easy bruisability; Danlos emphasized the pseudotumours. [1] Hypermobile joint, hyperelastic skin, associated with easy bruisability, fragility, and atrophic scarring are the major components of EDS. The 10 types of EDS have been described on account of clinical, biochemical, and hereditary hetero­geneity. [2],[3] Though most types of EDS are rare, EDS Type II(mitis), of which there is a mild subvariant, may be quite common. [4] We report one such case.


  Case Report Top


A 35-years-old man presented with hyper stretchable skin and scars over his bony prominences. The patient complained of difficulty in performing precision movement like writing on account of his hypermobile fingers. He could demonstrate his extreme loose-jointedness by crossing his fingers in several difficult position [Figure - 1]. On examination his skin could be stretched to a great extent, but it returned immediately to normal [Figure - 2]. Skin extensibility was maximal over arms, knees and elbows. Linear "cigarette paper" atrophic scars were found on his right elbow and both shins. There was no evidence of bruises, ecchymoses, ''molluscoid pseudotumours" or "calcified spherules". No clinical evidence of ocular or visceral involvement was present. No family history of similar abnormality could be elicited.


  Comments Top


The presence of joint hypermobility, increased skin extensibility and abnormal scarring were pointers to a diagnosis of EDS in our patient. Absence of ocular lesions, ecchymoses, large joint dislocations, and periodontitis led us to classify our patient as EDS Type II (mitis). To clinch our diagnosis we used a clinical scoring system as recommended by Holzberg et al, [4] as shown in [Table - 1].

Our patient scored a total of 8 points. A score of 7 identifies cases of mild EDS type IL [4] Identification of such cases may be important prognostically in patients with diseases in which collagen plays a major role, such as joint diseases (dislocations), bruising abnormalities (pigmented purpura), and potentially scarring diseases (acne or patients undergoing skin surgery) . [4]

Familial articular hypermobility was considered in the differential diagnosis of our patient. The hallmark of this disorder is hypermobility of joints in the absence of skin extensibility or tissue fragility. [5] The presence of extensible skin and scars in our patient excluded this possibility.

 
  References Top

1.Der Kaloustain VM, Kurban AK. Ehlers-Danlos Syndrome (Cutis Hyperelastica). In : Genetic Diseases of the Skin, 1st edn. New York Springer Verlag, 1979; 164-5.  Back to cited text no. 1    
2.Horton WA, Hood OJ. Ehlers-Danlos Syndrome. In : Birth Defects Encyclopedia (Buyse ML, ed), Blackwell Scientific Publications, 1990; 610-1.  Back to cited text no. 2    
3.Burton JL. Disorders of Connective Tissue. In : Textbook of Dermatology (Champion RH, Burton JL, Ebling FJG, eds), 5th edn. Oxford : Blackwell Scientific Publications, 1992; 1763-826.  Back to cited text no. 3    
4.Holzberg M, Hewan-Lowe KO, Olansky AJ. The Ehlers-Danlos Syndrome : recognition, characterization, and importance of a milder variant of the classic form. A preliminary study. J Am Acad Dermatol 1988; 19 : 656-66.  Back to cited text no. 4  [PUBMED]  
5.Winship IM. Articulai hypermobility, familial. In : Birth Defects Encyclopedia (Buyse ML, ed), Blackwell Scientific Publications 1990; 198-9.  Back to cited text no. 5    


    Figures

[Figure - 1], [Figure - 2]

    Tables

[Table - 1]

This article has been cited by
1 Ehlers-Danlos syndrome - Clinical presentation
Hingorjo, M.R.
Journal of the Pakistan Medical Association. 2008; 58(5): 279-281
[Pubmed]



 

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