Updated May 20, 2014.

Introduction

Morphea or localized scleroderma, is a rare inflammatory disease that primarily affects deep layers of the skin and fat, with an increase in collagen causing thickening of skin. The incidence of morphea ranges from 0.4 to 2.7 per 100,000 people. Morphea is more common in female than male patients at a ratio of around 3 to 4:1 and commonly affects adults. However, certain subtypes, such as linear morphea, are most common in children and adolescents.^1,2 Linear morphea and most commonly occurs around 2-14 years of age.^3,4 The most frequent type of morphea in adults, plaque morphea, most commonly occurs around 40-50 years of age.

In Part I of this article we will discuss how morphea is different from systemic scleroderma, the different subtypes of morphea, as well as the mechanism of this disease. In Part IIof this article, we discuss the presentation, testing, and treatment options.

Morphea versus systemic scleroderma

Scleroderma is a type of autoimmune fibrosing disorder. The names used for the types of scleroderma can be confusing. This article describes morphea, also called localized scleroderma, which is a fibrosing disorder that mainly affects the skin. It is important todistinguish morphea from systemic scleroderma or systemic sclerosis, which can involve the body’s internal organs. Some features that are suggestive of systemic scleroderma include: Raynaud’s phenomenon (causing color changes and discomfort of the fingers or toes), nailfold blood vessel changes, visible tiny blood vessels (telangiectasias), or sclerosis of the fingers and toes.^3,5 Only systemic sclerosis is associated with internal organ system involvement (cardiac, lung or gastrointestinal).⁴ The distribution of skin findings can also be helpful.

In morphea, skin involvement is often asymmetric, patchy, or linear and does not extend towards upper extremities or internal organs.

Classification

Morphea is categorized into five subtypes developed by Peterson et al.⁶ These categories include: plaque morphea, generalized morphea, bullous morphea, linear morphea, and deep morphea.

1) Plaque morphea (Figure 1), the most common subtype, often presents with defined, hardened plaques with a yellow-white center and a surrounding  red-purple colored halo. Plaque morphea often involves only one or two sites on the body. 

2) Generalized morphea is characterized by lesions on two or more parts of the body, which can grow together to form a larger plaque. Often, patients will have sites with multiple hardened plaques with darkened areas of pigmentation and/or muscle wasting.

3) Bullous morphea is characterized by tense, large blisters in the upper layers of the skin that may overlie morphea changes.

4) Linear morphea is characterized by band-like streaks that may be depressed on the skin. Linear morphea often occurs on the extremities or face and scalp and may also involve structures underneath the skin, including soft tissue, muscle, and bone.^7,8  Linear morphea often occurs in children.  The “en coup de sabre” subtype is common in childhood and often located on the forehead, extending into the scalp.  This subtype may be associated with underlying abnormalities which may cause seizures and headaches.

5) Deep morphea is the most rare subtype of morphea (<5% of patients affected with morphea). Deep morphea is characterized by many lesions involving structures under the skin including subcutaneous tissue, fascia, or superficial muscle.^4,6                   

Mechanism and Cause

The cause of morphea is unknown. It may be caused by multiple factors and could be associated with trauma, radiation, medications, infection, and autoimmunity.³ These factors may lead to small vessel damage, which prompts the release of signals that can cause hardening of structures in the skin.^3,4,9

 

References

1. Peterson LS, Nelson AM, Su WP, Mason T, O'Fallon WM, Gabriel SE. The epidemiology of morphea (localized scleroderma) in Olmsted County 1960-1993. The Journal of rheumatology 1997;24:73-80.

2. Murray KJ, Laxer RM. Scleroderma in children and adolescents. Rheum Dis Clin North Am 2002;28:603-24.

3. Fett N, Werth VP. Update on morphea: part I. Epidemiology, clinical presentation, and pathogenesis. Journal of the American Academy of Dermatology 2011;64:217-28; quiz 29-30.

4. Kreuter A. Localized scleroderma. Dermatol Ther 2012;25:135-47.

5. Hudson M, Taillefer S, Steele R, et al. Improving the sensitivity of the American College of Rheumatology classification criteria for systemic sclerosis. Clinical and experimental rheumatology 2007;25:754-7.

6. Peterson LS, Nelson AM, Su WP. Classification of morphea (localized scleroderma). Mayo Clin Proc 1995;70:1068-76.

7. Zulian F, Athreya BH, Laxer R, et al. Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study. Rheumatology 2006;45:614-20.

8. Zulian F, Vallongo C, Woo P, et al. Localized scleroderma in childhood is not just a skin disease. Arthritis and rheumatism 2005;52:2873-81.

9. Vasquez R, Sendejo C, Jacobe H. Morphea and other localized forms of scleroderma. Current opinion in rheumatology 2012;24:685-93.