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 .To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
- Provost TT, Greenberg AS, Falanga V. Localized cutaneous sclerosis. In: Cutaneous manifestations of rheumatic diseases, 1st ed, Sontheimer RD, Provost TT (Eds), Williams & Wilkins, Baltimore 1996. p.125.
- Sehgal VN, Srivastava G, Aggarwal AK, et al. Localized scleroderma/morphea. Int J Dermatol 2002; 41:467.
- Christen-Zaech S, Hakim MD, Afsar FS, Paller AS. Pediatric morphea (localized scleroderma): review of 136 patients. J Am Acad Dermatol 2008; 59:385.
- Leitenberger JJ, Cayce RL, Haley RW, et al. Distinct autoimmune syndromes in morphea: a review of 245 adult and pediatric cases. Arch Dermatol 2009; 145:545.
- Peterson LS, Nelson AM, Su WP, et al. The epidemiology of morphea (localized scleroderma) in Olmsted County 1960-1993. J Rheumatol 1997; 24:73.
- Dharamsi JW, Victor S, Aguwa N, et al. Morphea in adults and children cohort III: nested case-control study--the clinical significance of autoantibodies in morphea. JAMA Dermatol 2013; 149:1159.
- Badea I, Taylor M, Rosenberg A, Foldvari M. Pathogenesis and therapeutic approaches for improved topical treatment in localized scleroderma and systemic sclerosis. Rheumatology (Oxford) 2009; 48:213.
- Grabell D, Hsieh C, Andrew R, et al. The role of skin trauma in the distribution of morphea lesions: a cross-sectional survey of the Morphea in Adults and Children cohort IV. J Am Acad Dermatol 2014; 71:493.
- Saxton-Daniels S, Jacobe HT. An evaluation of long-term outcomes in adults with pediatric-onset morphea. Arch Dermatol 2010; 146:1044.
- Takehara K, Moroi Y, Nakabayashi Y, Ishibashi Y. Antinuclear antibodies in localized scleroderma. Arthritis Rheum 1983; 26:612.
- Saracino AM, Denton CP, Orteu CH. The molecular pathogenesis of morphoea: from genetics to future treatment targets. Br J Dermatol 2016.
- Gabrielli A, Avvedimento EV, Krieg T. Scleroderma. N Engl J Med 2009; 360:1989.
- Ihn H, Sato S, Fujimoto M, et al. Demonstration of interleukin-2, interleukin-4 and interleukin-6 in sera from patients with localized scleroderma. Arch Dermatol Res 1995; 287:193.
- Fett N, Werth VP. Update on morphea: part I. Epidemiology, clinical presentation, and pathogenesis. J Am Acad Dermatol 2011; 64:217.
- Yamane K, Ihn H, Kubo M, et al. Increased serum levels of soluble vascular cell adhesion molecule 1 and E-selectin in patients with localized scleroderma. J Am Acad Dermatol 2000; 42:64.
- Wadud MA, Bose BK, Al Nasir T. Familial localised scleroderma from Bangladesh: two case reports. Bangladesh Med Res Counc Bull 1989; 15:15.
- REES RB, BENNETT J. Localized scleroderma in father and daughter. AMA Arch Derm Syphilol 1953; 68:360.
- Zulian F, Vallongo C, Woo P, et al. Localized scleroderma in childhood is not just a skin disease. Arthritis Rheum 2005; 52:2873.
- Laxer RM, Zulian F. Localized scleroderma. Curr Opin Rheumatol 2006; 18:606.
- Zulian F, Athreya BH, Laxer R, et al. Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study. Rheumatology (Oxford) 2006; 45:614.
- Marzano AV, Menni S, Parodi A, et al. Localized scleroderma in adults and children. Clinical and laboratory investigations on 239 cases. Eur J Dermatol 2003; 13:171.
- Vierra E, Cunningham BB. Morphea and localized scleroderma in children. Semin Cutan Med Surg 1999; 18:210.
- Patel AR, Pavletic SZ, Turner ML, Cowen EW. The isomorphic response in morphealike chronic graft-vs-host disease. Arch Dermatol 2008; 144:1229.
- Minato H, Taki R, Miyachi Y, Utani A. Symmetrical pigmented sclerosis enclosed by pruritic erythema: a new variant of morphoea? Br J Dermatol 2009; 161:703.
- Weibel L, Harper JI. Linear morphoea follows Blaschko's lines. Br J Dermatol 2008; 159:175.
- Muroi E, Ogawa F, Yamaoka T, et al. Case of localized scleroderma associated with osteomyelitis. J Dermatol 2010; 37:81.
- Horger M, Fierlbeck G, Kuemmerle-Deschner J, et al. MRI findings in deep and generalized morphea (localized scleroderma). AJR Am J Roentgenol 2008; 190:32.
- Dehen L, Roujeau JC, Cosnes A, Revuz J. Internal involvement in localized scleroderma. Medicine (Baltimore) 1994; 73:241.
- Zulian F. New developments in localized scleroderma. Curr Opin Rheumatol 2008; 20:601.
- Tollefson MM, Witman PM. En coup de sabre morphea and Parry-Romberg syndrome: a retrospective review of 54 patients. J Am Acad Dermatol 2007; 56:257.
- Zannin ME, Martini G, Athreya BH, et al. Ocular involvement in children with localised scleroderma: a multi-centre study. Br J Ophthalmol 2007; 91:1311.
- Chen JK, Chung L, Fiorentino DF. Characterization of patients with clinical overlap of morphea and systemic sclerosis: A case series. J Am Acad Dermatol 2016; 74:1272.
- Walker D, Susa JS, Currimbhoy S, Jacobe H. Histopathological changes in morphea and their clinical correlates: Results from the Morphea in Adults and Children Cohort V. J Am Acad Dermatol 2017; 76:1124.
- Falanga V, Medsger TA Jr, Reichlin M. Antinuclear and anti-single-stranded DNA antibodies in morphea and generalized morphea. Arch Dermatol 1987; 123:350.
- Arkachaisri T, Fertig N, Pino S, Medsger TA Jr. Serum autoantibodies and their clinical associations in patients with childhood- and adult-onset linear scleroderma. A single-center study. J Rheumatol 2008; 35:2439.
- Rosenberg AM, Uziel Y, Krafchik BR, et al. Antinuclear antibodies in children with localized scleroderma. J Rheumatol 1995; 22:2337.
- Sato S, Kodera M, Hasegawa M, et al. Antinucleosome antibody is a major autoantibody in localized scleroderma. Br J Dermatol 2004; 151:1182.
- Sato S, Ihn H, Soma Y, et al. Antihistone antibodies in patients with localized scleroderma. Arthritis Rheum 1993; 36:1137.
- Parodi A, Drosera M, Barbieri L, Rebora A. Antihistone antibodies in scleroderma. Dermatology 1995; 191:16.
- Tomimura S, Ogawa F, Iwata Y, et al. Autoantibodies against matrix metalloproteinase-1 in patients with localized scleroderma. J Dermatol Sci 2008; 52:47.
- Kroft EB, de Jong EM, Evers AW. Psychological distress in patients with morphea and eosinophilic fasciitis. Arch Dermatol 2009; 145:1017.
- Schanz S, Fierlbeck G, Ulmer A, et al. Localized scleroderma: MR findings and clinical features. Radiology 2011; 260:817.
- Li SC, Liebling MS, Haines KA. Ultrasonography is a sensitive tool for monitoring localized scleroderma. Rheumatology (Oxford) 2007; 46:1316.
- Wortsman X, Wortsman J, Sazunic I, Carreño L. Activity assessment in morphea using color Doppler ultrasound. J Am Acad Dermatol 2011; 65:942.
- Morganroth PA, Dehoratius D, Curry H, Elenitsas R. Postirradiation Morphea: A Case Report With a Review of the Literature and Summary of the Clinicopathologic Differential Diagnosis. Am J Dermatopathol 2013.
- Dubner S, Bovi J, White J, Susnik B. Postirradiation morphea in a breast cancer patient. Breast J 2006; 12:173.
- Fruchter R, Kurtzman DJ, Mazori DR, et al. Characteristics and treatment of postirradiation morphea: A retrospective multicenter analysis. J Am Acad Dermatol 2017; 76:19.
- Lutz V, Francès C, Bessis D, et al. High frequency of genital lichen sclerosus in a prospective series of 76 patients with morphea: toward a better understanding of the spectrum of morphea. Arch Dermatol 2012; 148:24.
- Falanga V, Killoran CE. Morphea. In: Fitzpatrick's dermatology in general medicine, 7th ed, Woff K, Goldsmith LA, Katz SI, et al. (Eds), McGraw-Hill, 2008. p.543.
- Castanet J, Lacour JP, Perrin C, et al. Association of eosinophilic fasciitis, multiple morphea and antiphospholipid antibody. Dermatology 1994; 189:304.
- Heidary N, Cheung W, Wang N, et al. Eosinophilic fasciitis/generalized morphea overlap. Dermatol Online J 2009; 15:2.
- Moulin C, Cavailhes A, Balme B, Skowron F. Morphoea-like plaques revealing an eosinophilic (Shulman) fasciitis. Clin Exp Dermatol 2009; 34:e851.
- 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