Types of Scleroderma: Overview

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Two Main Types: Localized and Systemic
Juvenile Scleroderma
Localized (Morphea, Linear, En Coup)
Nodular Scleroderma
Systemic Sclerosis (Limited/CREST, Diffuse, Overlap)


Types of SclerodermaTypes of Scleroderma
Presented by Isabella Ensz

There are many methods used to categorize the various forms of scleroderma, which has brought about a confusing abundance of terms.

The following categories are often found in patient literature, but use and interpretation of these categories varies quite a bit. (Also see What is Scleroderma?)

Two Main Types

There are two main types of scleroderma: Localized and Systemic.

The systemic forms can affect any part of the body (skin, blood vessels, and internal organs.) The systemic forms are also referred to as "systemic sclerosis" plus other terms such as diffuse, limited, CREST, and overlap.

The localized forms are morphea and linear. They affect the skin (and sometimes the underlying muscles or bones), and occasionally cause a few other symptoms.

When any form of scleroderma (either localized or systemic) occurs in children, it is also called Childhood Scleroderma or Juvenile Scleroderma.

Juvenile Scleroderma

Juvenile Scleroderma. When any form of scleroderma (either localized or systemic) occurs in children, it is also called Childhood Scleroderma or Juvenile Scleroderma. ISN.

Coping with a Child's Illness
Patient and Caregiver Stories


Localized Scleroderma.There are two types of localized scleroderma: linear and morphea. Localized scleroderma affects the skin. It may also affect the underlying muscles and bones, but it does not affect internal organs, and it does not reduce a person's life expectancy. ISN.

Extracutaneous Symptoms
Patient and Caregiver Stories

Nodular Systemic Scleroderma

Case Report: Scleroderma with Nodular Scleroderma. Although this condition is rare, it has been reported sporadically, and clinicians should be able to recognize this variant in cases of scleroderma presenting with firm nodules or plaques. PubMed, Case Rep Dermatol.

(Expired Article) Nodular Scleroderma: Case Report and Literature Review. Nodular Systemic Sclerosis is a rare variant that presents with lesions that clinically resemble keloids. Most patients had symptoms of systemic sclerosis. J Rheumatol. (Also see Diseases Similar to Scleroderma)

Systemic Sclerosis (Systemic Scleroderma)

Systemic scleroderma is also known as systemic sclerosis (SSc). There are several types, Limited/CREST and Diffuse. These types can affect the entire body, including the skin, blood vessels, and/or internal organs. ISN.

Types of Systemic Scleroderma
Classification: Limited and Diffuse
CREST (old name for Limited)
Limited Systemic Sclerosis
Diffuse Systemic Sclerosis

Systemic Sclerosis: Prognosis and Mortality

Systemic Sclerosis (Scleroderma): Prognosis and Mortality. Now that the full range of types and severity of scleroderma are being recognized and diagnosed, there is increasing awareness that many people have a milder type of illness with a much rosier longterm prognosis. ISN.

Age at Onset
Fear of Progression
Cardiac Involvement
Causes of Death
Lung Involvement
Nailfold Indicators
Prognostic Markers
Skin Thickness Progression
Symptoms of Systemic Scleroderma

Also See

Juvenile Scleroderma by ISN.

Scleroderma Patient and Caregiver Stories by ISN.

Go to Localized: Linear Scleroderma

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