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DOI: https://doi.org/10.22263/2312-4156.2017.6.107

Adaskevich U.P., Marozava A.U.
Classical type of Ehlers-Danlos syndrome in a 56-year-old patient
Vitebsk State Order of Peoples’ Friendship Medical University, Vitebsk, Republic of Belarus

Vestnik VGMU. 2017;16(6):107-113.

Abstract.
The Ehlers-Danlos syndrome (EDS) is the name given to a clinically and genetically heterogeneous group of heritable connective tissue disorders characterized by joint hypermobility, skin hyperelasticity, and tissue fragility.
A new international classification of EDS is presented herein with a detailed description of 13 syndrome types and major and minor criteria suggested for the disease diagnosis.
A rare case of a heritable connective tissue disorder – a classical EDS type is described in a 56-year-old male patient. The examination of the patient’s integument revealed a soft, doughy, highly stretchy character of the skin which could be easily folded. Abundant wide atrophic scars were observed on the face (forehead and cheeks), the upper and lower extremities. The skin in these areas was extremely thin and had a «cigarette paper» appearance. Persisting hematomas and insignificant edema, indolent on palpation, were seen in the area of proximal interphalangeal joint on the left hand second finger as well as in that of both knee joints.
The Ehlers-Danlos syndrome is a rare hereditary disease the manifestations of which can involve several organs and systems of the body. The patient’s quality of life and working ability in this case will greatly depend on a careful medical examination and timely and correct diagnosis.
Key words: Ehlers-Danlos syndrome, classification, diagnostic criteria.

References

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Information about authors:
Adaskevich U.P. – Doctor of Medical Sciences, professor, head of the Chair of Dermatovenerology, Vitebsk State Order of Peoples’ Friendship Medical University;
Marozava A.U. – resident of the Chair of Dermatovenerology, Vitebsk State Order of Peoples’ Friendship Medical University.

Correspondence address: Republic of Belarus, 210023, Vitebsk, 27 Frunze ave., Vitebsk State Order of Peoples’ Friendship Medical University, Chair of Dermatovenerology. E-mail: Этот адрес электронной почты защищён от спам-ботов. У вас должен быть включен JavaScript для просмотра. – Uladzimir P. Adaskevich.

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