Joint hypermobility and skin elasticity: the hereditary disorders of connective tissue.

Abstract:

:The hereditary disorders of connective tissues (HDCTs) encompass a spectrum of conditions linked pathophysiologically by abnormalities of collagen, fibrillin, and matrix proteins. The clinical picture ranges from morbidity because of musculoskeletal, skin, ocular and visceral pathologies to mortality from acute vascular collapse. For many of the conditions, there is a considerable overlap in clinical features, although severity varies; appreciating the subtle differences in presentation is vital to the clinician in determining the diagnosis. Though conditions associated with severe vascular pathology are rare, other hereditary disorders of connective tissues such as the joint hypermobility syndrome and Stickler's disease are common and probably underrecognized. Abnormal skin elasticity and scaring, joint hypermobility, and chronic arthralgia are important clues that should trigger the clinician to search for underlying hereditary disorders of connective tissues. In this article, we discuss the spectrum of clinical findings, management, and genetic screening of the more common hereditary disorders of connective tissues, highlighting their diagnostic criteria and their differences.

journal_name

Clin Dermatol

journal_title

Clinics in dermatology

authors

Hakim AJ,Sahota A

doi

10.1016/j.clindermatol.2006.07.013

subject

Has Abstract

pub_date

2006-11-01 00:00:00

pages

521-33

issue

6

eissn

0738-081X

issn

1879-1131

pii

S0738-081X(06)00106-4

journal_volume

24

pub_type

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