Morphea: Current concepts
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:
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Evidence of clinical activity of the disease
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Depth of lesion involvement
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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
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Ms. Florez-Pollack and Ms. Kunzler are co-first authors.