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Cutaneous Manifestations of Scleroderma and Scleroderma-Like Disorders: a Comprehensive Review

Clinical Reviews in Allergy & Immunology volume 53, pages 306–336 (2017)Cite this article

Abstract

Scleroderma refers to an autoimmune connective tissue fibrosing disease, including three different subsets: localized scleroderma, limited cutaneous systemic sclerosis, and diffuse cutaneous systemic sclerosis with divergent patterns of organ involvement, autoantibody profiles, management, and prognostic implications. Although systemic sclerosis is considered the disease prototype that causes cutaneous sclerosis, there are many other conditions that can mimic and be confused with SSc. They can be classified into immune-mediated/inflammatory, immune-mediated/inflammatory with abnormal deposit (mucinoses), genetic, drug-induced and toxic, metabolic, panniculitis/vascular, and (para)neoplastic disorders according to clinico-pathological and pathogenetic correlations. This article reviews the clinical presentation with emphasis on cutaneous disease, etiopathogenesis, diagnosis, and treatment options available for the different forms of scleroderma firstly and for scleroderma-like disorders, including scleromyxedema, scleredema, nephrogenic systemic fibrosis, eosinophilic fasciitis, chronic graft-versus-host disease, porphyria cutanea tarda, diabetic stiff-hand syndrome (diabetic cheiroartropathy), and other minor forms. This latter group of conditions, termed also scleroderma mimics, sclerodermiform diseases, or pseudosclerodermas, shares the common thread of skin thickening but presents with distinct cutaneous manifestations, skin histology, and systemic implications or disease associations, differentiating each entity from the others and from scleroderma. The lack of Raynaud’s phenomenon, capillaroscopic abnormalities, or scleroderma-specific autoantibodies is also important diagnostic clues. As cutaneous involvement is the earliest, most frequent and characteristic manifestation of scleroderma and sclerodermoid disorders, dermatologists are often the first-line doctors who must be able to promptly recognize skin symptoms to provide the affected patient a correct diagnosis and appropriate management.

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Abbreviations

SSc:

Systemic sclerosis

LSc:

Localized scleroderma

lcSSc:

Limited cutaneous systemic scleroderma

dcSSc:

Diffuse cutaneous systemic scleroderma

Anti-RNAP III:

anti-RNA polymerase III

TGF-ß:

Transforming growth factor-ß

TNF:

Tumor necrosis factor

Fli1:

Friend leukemia integration factor 1

PDGF:

Platelet-derived growth factor

ACR:

American College of Rheumatology

EULAR:

European League Against Rheumatism

EUSTAR:

European Scleroderma Trials and Research

ANA:

Anti-nuclear antibody

MES:

Microangiopathy evolutiontion score

NVC:

Nailfold videocapillaroscopy

CT:

Cutaneous telangectasias

mRSS:

Modified Rodnan skin score

DUs:

Digital ulcers

ENA:

Extractable nuclear antigens

ACA:

Anti-centromere antibody

Anti-Scl-70:

Anti-topoisomerase I

PM/Scl:

Polymiositis/scleroderma

PFH:

Progressive hemifacial atrophy

LoSSI:

Localized scleroderma Skin Severity Index

MTX:

Methotrexate

NSF:

Nephrogenic systemic fibrosis

GBCAs:

Gadolinium-based contrast agents

MRI:

Magnetic resonance imaging

EF:

Eosinophilic fasciitis

HLA:

Human leukocyte antigens

IFN gamma:

Interferon gamma

TIMP-1:

Metalloproteinase

ESR:

Erythrocyte sedimentation rate

PET-CT:

Positron emission tomography

GVHD:

Graft-versus-host disease

La/SSB:

Anti-lupus anti-coagulant Sjögren’s syndrome-related antigen B

PUVA:

Psoralen plus ultraviolet A

GVT:

Graft versus tumor

PCT:

Porphyria cutanea tarda

UROD:

Uroporphyrinogen decarboxylase

HFE:

Hemochromatosis

HIV:

Human immunodeficiency virus

URO:

Uroporphyrin

COPRO:

Coproporphyrin

ALA:

Aminolevulinic acid

PBG:

Porphobilinogen

DM:

Diabetes mellitus

AGEs:

Advanced glycosylation end-products

WS:

Werner syndrome

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Authors and Affiliations

  1. Section of Dermatology, Department of Medical Sciences and Public Health, University of Cagliari, Cagliari, Italy

    Caterina Ferreli, Franco Rongioletti & Laura Atzori

  2. Section of Dermatology, Department of Health Sciences, DISSAL, IRCSS-AOU S. Martino-IST, University of Genoa, Genoa, Italy

    Giulia Gasparini, Aurora Parodi & Emanuele Cozzani

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  1. Caterina Ferreli
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  2. Giulia Gasparini
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  3. Aurora Parodi
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  4. Emanuele Cozzani
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Correspondence to Caterina Ferreli.

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Ferreli, C., Gasparini, G., Parodi, A. et al. Cutaneous Manifestations of Scleroderma and Scleroderma-Like Disorders: a Comprehensive Review. Clinic Rev Allerg Immunol 53, 306–336 (2017). https://doi.org/10.1007/s12016-017-8625-4

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Keywords

  • Scleroderma
  • Systemic sclerosis
  • Morphea
  • Scleroderma-like disorders
  • Cutaneous sclerosis