Localized Scleroderma: Linear

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Video Overview of Linear
Blaschko's Lines
En Coup de Sabre
Juvenile Scleroderma
Parry Rombergs Syndrome
Sclerodermic Linear Lupus Panniculitis
Linear Patient Stories
Also See

Video Overview of Linear Scleroderma

Overview of Linear Scleroderma Video
Video: Overview of Linear Scleroderma

Presented by Amanda Thorpe
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Proper diagnosis of scleroderma is often long and difficult, since it is a such a rare disease, and one which few doctors or patients are well-versed in.

Linear scleroderma is a line of thickened skin which can affect the bones and muscles underneath it, thus limiting the motion of the affected joints and muscles. It most often occurs in the arms, legs, or forehead, and may occur in more than one area.

It is most likely to be on just one side of the body. Linear scleroderma generally appears in young children, and is characterized by the failure of one arm or leg to grow as rapidly as its counterpart. (Also see Types of Scleroderma)

Autoimmune Disorder

Localized scleroderma is an autoimmune disorder. Many previous studies conclude that localized scleroderma involves autoimmune abnormalities and is one of the organ-specific autoimmune disorders targeting mainly skin, although the types of autoimmune abnormality are different from systemic sclerosis. Rheumatology (Oxford). Vol 44. No. 3. 2004 Nov 23. (Also see Types of Scleroderma: Localized, Morphea and Dr. Shinichi Sato)

Blaschko's Lines

Linear morphoea follows Blaschko's lines. Our data indicate that linear morphoea follows the lines of Blaschko. We hypothesize that in patients with linear morphoea susceptible cells are present in a mosaic state and that exposure to some trigger factor may result in the development of this condition. (PubMed) Br J Dermatol. 2008 Jul;159(1):175-81. (Also see Morphea)

Diagnosis of Linear Scleroderma

Diagnosis of Linear Scleroderma is usually done by clinical examination. It is often confirmed by skin biopsy. Skin biopsies are usually a very tiny sample of tissue and the procedure is often very quick, easy and with minimal discomfort. Ultrasound has also been found to be useful for the diagnosis of Localized Scleroderma (such as linear). ISN.

Juvenile Scleroderma Experts
Blood Tests
Computerized Skin Score (CSS)
Severity Index
Also See

En Coup de Sabre

En Coup de Sabre is a term used when linear scleroderma affects the forehead. It appears as an indented, vertical, colorless, line of skin on the forehead. The indentation looks as though the person may have been struck by a sword. ISN.

En Coup in Overlap
Patient Stories

Juvenile (Childhood) 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

Parry Rombergs Syndrome

Parry Rombergs Syndrome, is also known as Progressive Hemifacial Atrophy or HFA. It may be the same as Linear Scleroderma affecting the face. ISN.

Support Groups
Patient & Caregiver Stories

Sclerodermic Linear Lupus Panniculitis

Linear lupus panniculitis of the scalp presenting as alopecia along Blaschko's lines: a distinct variant of lupus panniculitis in East Asians? We propose linear lupus panniculitis of the scalp to be a distinct variant of lupus panniculitis and should be included in the differential diagnosis for focal or linear alopecia, especially in East Asians. J Dermatol, 2012 Apr;39(4):385-8.

Symptoms of Linear Scleroderma

Linear scleroderma is a line of thickened skin which can affect the bones and muscles underneath it, thus limiting the motion of the affected joints and muscles. It most often occurs in the arms, legs, or forehead, and may occur in more than one area.

A cross-sectional electromyography (EMG) assessment in linear scleroderma (LS) patients. In a small series of LS patients, there was a surprising amount of muscle dysfunction by EMG. The muscle involvement may be possibly related to a secondary peripheral nerve involvement due to LS inflammation and fibrosis. PubMed, Pediatr Rheumatol Online J, 2014 Jul 12;12:27.


Potential Scleroderma Gene Therapy Moving into Preclinical Dosing Study, Fibrocell Reports. Fibrocell has finished a proof-of-concept trial on FCX-013 for the treatment of linear scleroderma. The trial examined the efficacy of FCX-013 in reducing dermal thickness in fibrotic tissue. Scleroderma News Today, 02/09/2016.

Laparoscopic Sleeve Gastrectomy for Linear Scleroderma. This article documents the case of a 45-year-old female with a 35-year history of unilateral linear scleroderma, who subsequently developed morbid obesity of her contralateral side. Jason Kasza, MD. (Liebertonline). Journal of Laparoendoscopic & Advanced Surgical Techniques. May 2010, 20(4): 355-357.

Linear Patient and Caregiver Stories

AJ Miller: Linear Scleroderma I have had Linear Scleroderma since 1968, when I was 7 years old. I have never met anyone else with it…

Alicia: Linear Scleroderma My doctor said the people who are looking at me and seeing the scars are the ones who need healing, and that I should pray for them…

Alicia B: Localized Morphea with possible Parry Romberg Syndrome The doctor kept saying something had eaten away at all the fatty tissue and the muscles which left it just skin, blood, bone, and nerve so when you look at it, it looks like I have dirt on my face and a gaping hole to go with it…

More Linear Patient and Caregiver Stories…

Also See

Causes of Scleroderma. ISN.

Juvenile Scleroderma. ISN.

Scleroderma Photos. Pictures of scleroderma symptoms, including photos of Linear Scleroderma.

Go to Linear Scleroderma: En Coup de Sabre
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