Pathogenesis, clinical manifestations, and diagnosis of morphea (localized scleroderma) in adults
- Heidi Jacobe, MD
Heidi Jacobe, MD
- Associate Professor, Department of Dermatology
- University of Texas Southwestern
Morphea, also known as localized scleroderma, is an idiopathic, inflammatory disorder that causes sclerotic changes in the skin. Morphea is distinct from systemic sclerosis (scleroderma), an autoimmune connective tissue disorder characterized by acral or diffuse cutaneous sclerosis and frequent systemic manifestations. Use of the term morphea diminishes the likelihood of confusion between these two disorders, which can lead to unnecessary patient anxiety. (See "Overview of the clinical manifestations of systemic sclerosis (scleroderma) in adults".)
Patients with morphea present with single or multiple inflammatory or sclerotic plaques. Disease activity typically persists for three to six years; some patients develop more persistent or recurring involvement. Cosmetic disfigurement or functional impairments due to atrophy or contractures often remain after the resolution of active disease. (See "Treatment of morphea (localized scleroderma) in adults", section on 'Prognosis'.)
The epidemiology, pathogenesis, clinical manifestations, and diagnosis of morphea in adults will be reviewed here. The treatment and prognosis of morphea in adults and the features and management of morphea in children are discussed elsewhere. (See "Localized scleroderma in childhood".)
Morphea is a relatively uncommon disorder that affects adults and children [1-4]. The annual incidence of morphea was approximately 3 per 100,000 people in a population in the United States between 1960 and 1993 .
Although morphea can occur at any age, many patients (50 to 65 percent) develop the disease as adults [4-6]. In a nested case-control study of 110 patients with adult-onset morphea and 77 patients with juvenile-onset disease conducted at an academic medical center, the mean ages of disease onset were 45 years and 10 years, respectively . Females are more susceptible to morphea than males; in a retrospective study of 82 patients with morphea, the female to male ratio was 2.6 to 1 .
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- CLINICAL MANIFESTATIONS
- Lesion evolution
- - Circumscribed (plaque) morphea
- Deep morphea
- - Generalized morphea
- - Linear morphea
- En coup de sabre
- - Mixed morphea
- Extracutaneous manifestations
- Serum autoantibodies
- Other serum abnormalities
- Imaging studies
- DIFFERENTIAL DIAGNOSIS
- RELATED DISORDERS
- Lichen sclerosus
- Atrophoderma of Pasini and Pierini
- Eosinophilic fasciitis
- SUMMARY AND RECOMMENDATIONS