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

Clinical and laboratory features of systemic sclerosis (SSc) complicated with localized scleroderma (LSc). LSc and SSc have several common characteristics, including endothelial cell dysfunction, immune activation, and excess fibrosis of the skin, and there exist several SSc cases complicated with LSc during the course of SSc. PubMed, J Dermatol, 2015 Mar;42(3):283-7. (Also see Localized Scleroderma)

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. (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.

Case Reports: Sclerodermic linear lupus panniculitis. Lupus erythematosus panniculitis is a rare disease characterized by deep subcutaneous nodules, most commonly localized on the upper limbs and face. We present here the clinical characteristics, course and laboratory findings of 2 patients having linear lupus erythematosus panniculitis with localized scleroderma-like changes. PubMed, Dermatology. (Also seeCauses of Lipodystrophy)

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.

Trichoscopic Features of Linear Morphea on the Scalp. The description of dermoscopic features in Linear scleroderma en coup de sabre on the scalp may help in the early diagnosis of the disease. PubMed, Skin Appendage Disord, 2018 Jan;4(1):31-33.

Orthopedic complications of linear morphea: Implications for early interdisciplinary care. Despite the use of systemic immunosuppressive therapy in many patients, approximately half of patients with linear morphea of an extremity have orthopedic disease. PubMed, Pediatr Dermatol, 11/09/2017.


A 26–Year Experience with Microsurgical Reconstruction of Hemifacial Atrophy and Linear Scleroderma. Patients treated early in their disease course have immediate and sustained correction of their deformity, with slowing or in most cases cessation of the disease process. PubMed, Plast Reconstr Surg, 2018 Nov;142(5):1275-1283. (Also see Parry Rombergs Syndrome)

Fibrocell Submits Investigational New Drug (IND) Application for Scleroderma Treatment. Fibrocell Science, Inc. announced the submission of a IND Application with the U.S. Food and Drug Administration (FDA) for FCX-013, a gene therapy candidate for the treatment of moderate to severe localized scleroderma. Globe Newswire, 02/05/2018.

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.

Case Report: Characterization and Treatment of Unilateral Facial Muscle Spasm in Linear Scleroderma. We report a case of linear scleroderma associated with spasm of muscles innervated not only by the trigeminal but also by the facial nerve. PubMed, Tremor Other Hyperkinet Mov (N Y).

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.

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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