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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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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DOI: https://doi.org/10.1007/s12016-017-8625-4