Elsevier

Clinics in Dermatology

Volume 36, Issue 4, July–August 2018, Pages 475-486
Clinics in Dermatology

Morphea: Current concepts

https://doi.org/10.1016/j.clindermatol.2018.04.005Get rights and content

Abstract

Morphea is an inflammatory, sclerosing skin disorder that can involve the underlying soft tissues. Although the cause of morphea remains poorly investigated, genetic predisposition, immune dysregulation, and environmental factors have been implicated. Morphea is associated with cosmetic and functional sequelae, and internal organ involvement is rare. Early diagnosis and treatment are imperative to minimize damage such as limitation of range of motion. This review summarizes advances in diagnosis and treatment of morphea, allowing clinicians to better serve patients with this condition.

Introduction

Morphea, or localized scleroderma, is an autoimmune skin disorder characterized by inflammation and sclerosis of the skin and soft tissues. The estimated incidence of morphea is 0.4 to 2.7 per 100,000 people.[1], [2] Although the pathogenesis of morphea is poorly understood, recent studies suggest that genetic, immune, traumatic, and iatrogenic factors may play a role.[3], [4], [5]

Although different classification schemes exist, the most commonly accepted morphea subtypes include linear, generalized, plaque (circumscribed), and mixed.[6], [7] Linear is the most frequently encountered subtype in pediatric-onset morphea, whereas plaque and generalized are the predominant adult-onset subtypes.8 The clinical presentation of morphea varies depending on subtype, depth of involvement, and current stage of evolution of the lesions. Active morphea lesions present with inflammation in the form of erythema and induration, often accompanied by pain and pruritus. Inactive lesions reveal sclerosis and atrophy that can involve the epidermis, dermis, and subcutaneous tissue. Damage from unchecked activity in morphea can cause cosmetic sequelae such as hair loss and subcutaneous atrophy, as well as functional impairment, including joint contracture.[9], [10]

Morphea has been associated with systemic findings that include the musculoskeletal and nervous systems.9 Importantly, these extracutaneous manifestations are distinct from those found in systemic sclerosis.11 An experienced clinician, who is familiar with the disease, should confirm the diagnosis of morphea and initiate appropriate evaluation and treatment in a timely manner.

This study was approved by the Institutional Review Board at University of Texas Southwestern Medical Center, Dallas, TX.

Section snippets

Evaluation of the patient with morphea

A thorough history, physical examination, and review of systems should be collected in all patients with morphea. These efforts should focus on identifying the lesions as morphea, and subsequently determining activity and depth of involvement. This is followed by ascertaining signs of extracutaneous disease. The clinician needs not only to examine the lesions, but also to elicit the history of evolution of the lesions from patients to assess activity. Figure 1 details an evaluation algorithm

Role of diagnostic tests in morphea

The diagnosis of morphea typically rests upon clinical evaluation. Biopsy, however, should be used to exclude other competing diagnoses, such as cutaneous metastasis or malignancy, and to help differentiate morphea from other sclerosing conditions such as scleromyxedema or nephrogenic systemic fibrosis. Dermatoscopy may be helpful to differentiate erythema (indicating activity) from telangiectasia (indicating damage) in atrophic lesions (Figure 5). If lesions are new or enlarging, biopsy from

Role of imaging in morphea

MRI has received increasing interest as an objective outcome measure in morphea. Due to the high resolution of the images and ability to define depth and breadth of involvement as well as discern inflammation, sclerosis, and atrophy, MRI has the potential to be a useful adjunct to clinical examination. This is particularly true for patients who have suspected involvement extending to the subcutis, fascia, and muscle, where clinical examination may be limited. MRI may also be helpful for

Differential diagnosis of morphea

Because the morphology of morphea lesions can vary depending on the stage of disease progression, it is important to consider a variety of possibilities in the differential diagnosis of a lesion of morphea (Table). Local tissue trauma is a known precipitating factor for morphea lesions, and patients may confuse morphea lesions with trauma-induced lesions, such as hematomas, as early lesions can be violaceous and tender. Morphea may appear over areas of the skin that were previously affected by

Therapeutic approach for morphea

The therapeutic decision making for morphea is guided by three principles:

  • 1.

    Evidence of clinical activity of the disease

  • 2.

    Depth of lesion involvement

  • 3.

    Extent of the disease40

The primary task of the clinician is to establish evidence of activity based on the physical examination, with the aid of clinical photographs to track chronological changes, clinical scoring tools such as the LoSCAT, and, when indicated, imaging. When a diagnosis of active morphea has been established, treatment is guided by the

Management of disease damage

As treatments aimed at halting active disease drive the progression of the disease to an inactive state, damage (atrophy, pigmentary alteration, and functional impairment) tends to remain stable or increase even in the context of successful treatment58; therefore, the vast majority of treated patients do not have resolution of their lesions. Practitioners and patients may falsely construe increased or stable damage as treatment failure. For this reason, it is important to thoroughly educate

Conclusions

Morphea is a rare inflammatory disorder that evolves from erythematous and violaceous indurated plaques to hyperpigmented lesions with central sclerosis and eventually atrophy. Also known as “localized scleroderma,” morphea is frequently confused with systemic sclerosis by both patients and practitioners. It is important to differentiate these two disorders and to make the correct diagnosis, as morphea is limited to the skin and has rare and distinct internal organ involvement. The clinical

References (60)

  • W.R. Heymann

    Lipodermatosclerosis

    J Am Acad Dermatol

    (2009)
  • R. Khayat et al.

    Sclerodermatous changes in porphyria cutanea tarda: six cases

    Ann Dermatol Venereol

    (2013)
  • B.A. Zwischenberger et al.

    A systematic review of morphea treatments and therapeutic algorithm

    J Am Acad Dermatol

    (2011)
  • N. Strickland et al.

    Attitudes and trends in the treatment of morphea: a national survey

    J Am Acad Dermatol

    (2015)
  • P.-G. Sator et al.

    Medium-dose is more effective than low-dose ultraviolet A1 phototherapy for localized scleroderma as shown by 20-MHz ultrasound assessment

    J Am Acad Dermatol

    (2009)
  • A. Kreuter et al.

    A randomized controlled study of low-dose UVA1, medium-dose UVA1, and narrowband UVB phototherapy in the treatment of localized scleroderma

    J Am Acad Dermatol

    (2006)
  • D. Karpec et al.

    The impact of high-dose narrowband ultraviolet A1 on dermal thickness, collagen and matrix-metalloproteinases in animal model of scleroderma

    J Photochem Photobiol B Biol

    (2017)
  • M. Kerscher et al.

    Low-dose UVA1 phototherapy for treatment of localized scleroderma

    J Am Acad Dermatol

    (1998)
  • R. Vasquez et al.

    Recurrence of morphea after successful ultraviolet A1 phototherapy: a cohort study

    J Am Acad Dermatol

    (2014)
  • L.S. Peterson et al.

    The epidemiology of morphea (localized scleroderma) in Olmsted County 1960-1993

    J Rheumatol

    (1997)
  • R. Knobler et al.

    European Dermatology Forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 1: localized scleroderma, systemic sclerosis and overlap syndromes

    J Eur Acad Dermatol Venereol

    (2017)
  • H. Jacobe et al.

    Major histocompatibility complex class I and class II alleles may confer susceptibility to or protection against morphea: findings from the Morphea in Adults and Children cohort

    Arthritis Rheumatol

    (2014)
  • F. Zulian et al.

    Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study

    Rheumatology (Oxford)

    (2006)
  • S.C. Li et al.

    Development of consensus treatment plans for juvenile localized scleroderma: a roadmap toward comparative effectiveness studies in juvenile localized scleroderma

    Arthritis Care Res (Hoboken)

    (2012)
  • A. Lis-Swiety et al.

    Localized scleroderma: clinical and epidemiological features with emphasis on adulthood- versus childhood-onset disease differences

    J Eur Acad Dermatol Venereol

    (2017)
  • D. Condie et al.

    Comparison of outcomes in adults with pediatric-onset morphea and those with adult-onset morphea: a cross-sectional study from the morphea in adults and children cohort

    Arthritis Rheumatol

    (2014)
  • N.K. Klimas et al.

    Health-related quality of life in morphoea

    Br J Dermatol

    (2015)
  • N.M. Fett

    Morphea (localized scleroderma)

    JAMA Dermatol

    (2013)
  • L.A. Goldsmith et al.

    Fitzpatrick’s Dermatology in General Medicine

    (2012)
  • T. Arkachaisri et al.

    Development and initial validation of the localized scleroderma skin damage index and physician global assessment of disease damage: a proof-of-concept study

    Rheumatology (Oxford)

    (2010)
  • Cited by (69)

    • Measuring asymmetry in facial morphea via 3-dimensional stereophotogrammetry

      2023, Journal of the American Academy of Dermatology
    • Nevoid telangiectasia patches in a 17-year-old boy

      2022, JAAD Case Reports
      Citation Excerpt :

      The more modern and clinically appropriate classification, the ‘Pauda criteria’, divides morphea into circumscribed, generalized, linear, pansclerotic, and mixed subtypes. Each subtype has different clinical manifestations and degree of involvement of the subcutaneous soft tissues.4 The patient’s findings were consistent with generalized morphea.

    • Localized scleroderma

      2024, Dermatologie
    View all citing articles on Scopus
    1

    Ms. Florez-Pollack and Ms. Kunzler are co-first authors.

    View full text