Scleroderma linear L94.1

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 03.03.2023

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band-shaped morphea; linear circumscribed scleroderma; linear localized scleroderma; linear morphea

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Localized (non-systemic) scleroderma arranged in stripes or bands with chronic course, possibly lasting for years. It is similar to the plaque or morphaea type, but its clinical course is usually more severe: besides the skin and the suncutis, the deep fasciae may also be affected. Their shrinkage leads to impaired contour and function.

Sclerodermae en coup de sabre (morphea en coup des sabre) is thought to be a variant of linear circumstrictive scleroderma in the frontal region.

Hemifacial progressive atrophy(Parry-Romberg syndrome), is considered by some authors to be a particularly severe and extensive variant of linear morphea. However, others consider it to be an idiopathic, independent neuro-vegetative complex disease spanning multiple organ boundaries, with cutaneous sclerosis not being an obligatory symptom.

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Start in early or middle childhood; 2/3 of the patients are younger than 18 years at the beginning of the disease.

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Extermities, forehead, capillitium

Clinical features
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The clinical picture of linear morphea is variable depending on age, extent and location of the lesions. Onset often unspectacular with discrete nonsymptomatic, nonscaling erythema.

Over months (sometimes years), there is gradual induration of the lesions, initially without involvement of the skin appendages (follicles detectable intralesionally).

As the sclerosis progresses, both subcutaneous adipose tissue and possibly underlying fascia and muscle are involved. The areas adhere firmly to the underlying tissue. The fasciae are indurated, and may tightly surround the muscles at the extremities, but skeletal subalgae. Carpal tunnel syndrome may occur as a typical complication, if the lesion is appropriately localized. Often at this stage there is lesional atrophy of the skin appendages (follicular structures are absent).

If linear overlapping joint areas are affected, mobility restrictions may occur.

The maximum variant of the joint-spanning atrophic linearmorphea is the pansclerotic circumscriticalmorphea also called"disabling pansclerotic morphea".

Cutaneous features of linear circumstrictive scleroderma may also be detectable in Parry-Romberg syndrome.

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  1. Duymaz A et al. (2009) Parry-Romberg syndrome: facial atrophy and its relationship with other regions of the body. Ann Plast Surg 63:457-461.
  2. Echenne B et al. (2007) Parry Romberg syndrome and linear scleroderma en coup de sabre mimicking Rasmussen encephalitis. Neurology 69:2274.
  3. El-Kehdy J et al (2021) A review of Parry-Romberg syndrome. J Am Acad Dermatol 67:769-84.
  4. Mertens JS et al.(2015) Disease recurrence in localized scleroderma: a retrospective analysis of 344 patients with paediatric- or adult-onset disease. Br J Dermatol 172:722-728.
  5. Yamamoto N et al (2021) Linear morphea involving the underlying muscle. Clin Rheumatol 40):4767-4768.


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Last updated on: 03.03.2023