Systemic sclerosis: Current concepts of skin and systemic manifestations.

Abstract:

:Systemic sclerosis is an uncommon autoimmune connective tissue disease with multiorgan system involvement and significant associated morbidity and mortality. Cutaneous signs and clinical manifestations are of particular importance, as they may be recognized before systemic manifestations, allowing earlier risk stratification into the limited and diffuse cutaneous subtypes, as well as earlier initiation of treatment. Important cutaneous manifestations include Raynaud's phenomenon, digital ulcers, cutaneous sclerosis, calcinosis cutis, telangiectasias, pruritus, and dyspigmentation. Despite investigation of a wide variety of treatments, no FDA-approved pharmacologic therapies exist for systemic sclerosis, and data from high-quality studies are limited. In the following review, we will discuss skin-directed therapies. Although there is evidence to support specific treatments for Raynaud's phenomenon, digital ulcers, and cutaneous sclerosis, there are limited rigorous studies evaluating the treatment of other cutaneous signs and clinical manifestations. Additional randomized-controlled trials and large observational studies are necessary to develop future evidence-based treatment options.

journal_name

Clin Dermatol

journal_title

Clinics in dermatology

authors

Pearson DR,Werth VP,Pappas-Taffer L

doi

10.1016/j.clindermatol.2018.04.004

subject

Has Abstract

pub_date

2018-01-01 00:00:00

pages

459-474

issue

4

eissn

0738-081X

issn

1879-1131

pii

S0738-081X(18)30063-4

journal_volume

36

pub_type

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