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Update on morphea: Part I. Epidemiology, clinical presentation, and pathogenesis

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Morphea, also known as localized scleroderma, is a rare fibrosing disorder of the skin and underlying tissues. Morphea is differentiated from systemic sclerosis based on the absence of sclerodactyly, Raynaud phenomenon, and nailfold capillary changes. Patients with morphea commonly have systemic symptoms, such as malaise, fatigue, arthralgias, and myalgias, as well as positive autoantibody serologies. However, involvement of morphea is almost uniformly limited to those tissues derived from the mesoderm. The underlying pathogenesis of morphea is incompletely understood at this time, but ultimately results in an imbalance of collagen production and destruction.

Section snippets

Epidemiology

Key points

  1. The incidence of morphea is 0.4 to 2.7 per 100,000 people

  2. Morphea is more common in whites

  3. Morphea is more common in females

  4. The prevalence of morphea is equal in adults and children

Morphea is a rare fibrosing disorder of the skin and underlying tissues. Epidemiologic studies have reported its incidence as 0.4 to 2.7 per 100,000 people.1, 2 A female predominance of 2.4 to 4.2:1 has been reported.3, 4 Several retrospective reviews have been published in the last 5 years with similar epidemiologic

Classification

Key points

  1. Morphea classification systems are based on clinical phenotype

Morphea is classified based on clinical presentations. The landmark article of morphea classification was published in 1995 by Peterson et al7 (Table I). Peterson et al7 recommended five subclassifications of morphea: plaque (including morphea en plaque [Fig 1], guttate [Fig 2], atrophoderma of Pasini and Pierini, keloidal, and lichen sclerosis et atrophicus); generalized (involving more than 2 body areas [Fig 3]); bullous; linear

Clinical presentation

Key points

  1. Early lesions of morphea present as erythematous to dusky violaceous patches and plaques that resolve into sclerotic, hairless, anhidrotic plaques with varying amounts of postinflammatory hyperpigmentation

  2. The most common clinical subsets of morphea are plaque, linear, general, and pansclerotic

  3. Plaque morphea is the most common presentation in adults

  4. Linear morphea is the most common presentation in children and often presents with fibrosis of underlying tissues, resulting in additional morbidity

Clinical differentiation from systemic sclerosis

Key points

  1. The presence of Raynaud phenomenon, nailfold capillary changes, and sclerodactyly are the earliest findings in patients with systemic sclerosis

  2. Primary Raynaud phenomenon is not uncommon in the general population

  3. Secondary Raynaud phenomenon should be suspected when onset is after 30 years of age, there are accompanying nailfold capillary changes, or there is digital necrosis

When examining a patient with “scleroderma,” differentiating morphea from systemic sclerosis is the physician’s paramount

Histology

Key points

  1. Morphea and systemic sclerosis cannot be differentiated by histopathologic examination

  2. Early morphea and systemic sclerosis lesions have lymphocytic perivascular infiltration in the reticular dermis and swollen endothelial cells

  3. Late morphea and systemic sclerosis lesions are characterized by thickened collagen bundles infiltrating the entire dermis and extending into the subcutaneous fat

  4. Loss of eccrine glands and blood vessels and “fat trapping” are common histologic findings in late morphea and

Systemic manifestations

Key points

  1. Extracutaneous manifestations in morphea are not rare

  2. The most common extracutanous manifestation is arthralgia

  3. Central nervous system fibrosis is most common in those children with head and neck involvement

  4. Children with head and neck morphea should have regular ophthalmologic examinations to monitor for asymptomatic involvement that may lead to irreversible damage (level III evidence)

  5. Patients with morphea are likely to have a family history of autoimmunity

Extracutaneous manifestations of morphea

Pathogenesis

Key points

  1. The underlying etiology of morphea is unknown

  2. Development of morphea likely requires an underlying predisposition and an environmental encounter

  3. Proposed environmental factors include trauma, medications, and infections

  4. Proposed host factors include autoimmunity and microchimerism

The underlying etiology of morphea remains elusive. The development of morphea is likely a multifactorial process. The following factors have all been associated with the formation of morphea: trauma/radiation,

Molecular pathogenesis

Key points

  1. Morphea lesions may be initiated by vascular injury

  2. The vascular injury increases expression of adhesion molecules, which recruit inflammatory cells

  3. The recruited inflammatory cells increase production of profibrotic cytokines, such as transforming growth factor–beta

  4. The profibrotic cytokines increase collagen production and decrease collagen destruction, resulting in an overabundance of collagen deposition

Both morphea and systemic sclerosis are pathologically defined by an increase in collagen

Conclusions

Morphea is a rare, clinically heterogeneous disease process defined by increased collagen deposition. Morphea is distinguished from systemic sclerosis by its lack of sclerodactyly, Raynaud phenomenon, and nailfold capillary changes. Central nervous system fibrosis most commonly affects those children with head and neck involvement. Children with head and neck morphea should have regular ophthalmologic examinations to monitor for asymptomatic involvement that may lead to irreversible damage if

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    Supported in part by a Merit Review Grant from the Department of Veterans Affairs Veterans Health Administration, Office of Research and Development, Biomedical Laboratory Research and Development, and by the National Institutes of Health (grant NIH K24-AR 02207) to Dr Werth.

    This article does not reflect the views of the VHA or the US Government.

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