PatientPlus articles are written by UK doctors and are based on research evidence, UK and European Guidelines. They are designed for health professionals to use, so you may find the language more technical than the condition leaflets.
Synonyms: localised scleroderma, morphea
Morphoea, or localised scleroderma, is a disorder of excessive collagen deposition leading to thickening of the dermis and sometimes the subcutaneous tissues. The word scleroderma means hard skin, and in localised scleroderma fat, fascia, muscle and bone may also be affected, but not internal organs as in systemic scleroderma. See the separate article Systemic Sclerosis (Scleroderma) for further information about this condition.
Morphoea is an uncommon condition where the patient has areas of thickened skin. Studies of incidence and prevalence are inadequate, and the actual numbers are likely to be far higher because many cases may not come to medical attention. However, a relatively recent study reports incidence in children (under age 16) in the UK and Ireland to be 3.4 per million children per year.
Up to half of all cases occur in children. Children are 90% more likely to have localised scleroderma than the systemic disease. The most common age of disease onset in children is 5-10 years. Women are at least twice as likely as men to be affected.
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In most cases the cause of morphoea is unknown. It is thought that a genetic predisposition may lead to an immunologically mediated cytokine release, causing inflammation, dysfunction of the connective tissue metabolism and subsequent fibrosis. It appears to be debatable whether there is any risk of progression to systemic sclerosis, but it is probably a completely separate disease.
It has been known to follow:
- Tick bites - it has been associated with Lyme disease.
- Measles and other viral infections.
- Localised injury.
- Autoimmune diseases including vitiligo, diabetes, lichen sclerosus and lichen planus.
- Radiotherapy. It may be as common as 2 in every 1,000 patients treated with radiotherapy.
A few familial cases have been reported.
- Plaque morphoea:
- This is the most common type of morphoea.
- Plaques are thickened, usually oval patches of skin between 1-20 cm (or greater) in diameter.
- They start as a mauve colour, then change to ivory white in the middle with a lilac edge over several months. Long-standing plaques may be brown.
- Their surface is hairless, smooth and shiny. They tend not to sweat. Several asymmetrical plaques may be present on both sides of the trunk and limbs.
- Sometimes the surface is hyperpigmented with very little to feel.
- Superficial morphoea:
- Middle-aged women usually present with symmetrical mauve-coloured patches in the skin folds.
- They are particularly common in the groin, armpits and under the breasts.
- Linear morphoea:
- This is the most common form in children.
- This may present on the scalp/forehead and limbs. This is most often found on the limb of a child.
- A long and narrow plaque may be associated with underlying contractures.
- A deep form affecting the scalp is called 'en coup de sabre' - a sabre cut. The hair is lost permanently and the underlying skull bone may shrink.
- Pansclerotic disabling morphoea:
- This is rare.
- It affects children and results in extensive hardening of skin and underlying muscle.
- The growth of bones may be affected.
- It can lead to joint contractures, non-healing ulcers and squamous cell carcinoma.
- Generalised morphoea:
- Four or more plaques affecting two or more body regions. There may be widespread skin thickening over the trunk.
- Parry-Romberg syndrome is a rare neurocutaneous disorder in which linear scleroderma is associated with progressive facial hemiatrophy, migraine, facial pain and epilepsy.
- Atrophoderma of Pierini and Pasini:
- Depression in the skin caused by significant loss of subcutaneous tissue.
It is possible for several types to be present in one patient.
Blood tests have little role in assessment of morphoea, although confirmatory tests are sometimes done to assist in diagnosis. Polyclonal increases in immunoglobulin G and M may occur, especially in patients with linear and deep morphoea. Autoantibodies (eg, rheumatoid factor, antinuclear antibodies) are frequently positive.
Although a presumptive diagnosis can frequently be made on clinical findings, a biopsy can be used to confirm the diagnosis and delineate the depth of involvement:
- For plaque-type and generalised morphoea, a deep punch biopsy (including subcutaneous fat) is usually sufficient.
- For linear and deep morphoea, an incisional biopsy extending down to muscle is required.
Radiography may be helpful in cases of linear or deep morphoea where involvement of the underlying bone is suspected. It can also be used to monitor paediatric patients for potential growth defects.
Ultrasound is increasingly useful to monitor underlying disease activity.
- Infiltrating secondary neoplasia is a rare possibility.
- A white atrophic surface can also be due to lichen sclerosus et atrophicus.
- Brown pigmentation on the surface is most likely to be due to post-inflammatory hyperpigmentation rather than morphoea.
All suspected cases should be referred for diagnosis.
Unfortunately there is no available, effective treatment for most cases of morphoea. Although several have shown possible benefit in research, few controlled trials have been performed. Therapy aimed at reducing inflammatory activity in early disease is more successful than attempts to decrease sclerosis in well-established lesions.
- Ultraviolet B (UVB) phototherapy for superficial skin involvement.
- UVA phototherapy for deeper skin involvement. Efficacy has been proven, although there is no certainty yet about the long-term safety and risk of skin cancer.
- Methotrexate +/- systemic corticosteroids for involvement of underlying tissues, and for rapidly progressive or severe disease.
- Topical tacrolimus.
- Topical calcipotriol.
Physiotherapy may be helpful to prevent joint contractures in severe disease.
- Generally the lesions gradually improve over a period of years and may even resolve spontaneously.
- Plaque-type morphoea is usually active for several years then slowly softens, leaving brown staining and sometimes depressed areas of skin.
- Linear morphoea tends to be more progressive and lasts longer but also eventually improves, although sometimes deposits of calcium arise within the lesions. It needs monitoring, as there may be reactivation after long dormant periods. There is a high likelihood of cosmetic sequelae.
- Limbs affected by severe morphoea may be stiff and weak if there is muscle wasting. Growth may be affected.
- One study found that morphoea which develops in childhood has a relapsing and remitting course and produces more severe lesions than that which arises for the first time in adulthood.
- Localised scleroderma is rarely life-threatening but may adversely affect quality of life, as it can be disfiguring and disabling.
Further reading & references
- Badea I, Taylor M, Rosenberg A, et al; Pathogenesis and therapeutic approaches for improved topical treatment in localized scleroderma and systemic sclerosis. Rheumatology (Oxford). 2008 Nov 20.
- Valanciene G, Jasaitiene D, Valiukeviciene S; Pathogenesis and treatment modalities of localized scleroderma. Medicina (Kaunas). 2010;46(10):649-56.
- Fett NM; Morphea (localized scleroderma). JAMA Dermatol. 2013 Sep;149(9):1124. doi: 10.1001/jamadermatol.2013.5079.
- Herrick AL, Ennis H, Bhushan M, et al; Incidence of childhood linear scleroderma and systemic sclerosis in the UK and Ireland. Arthritis Care Res (Hoboken). 2010 Feb;62(2):213-8. doi: 10.1002/acr.20070.
- Childhood Onset Scleroderma; Scleroderma Society
- Weibel L, Laguda B, Atherton D, et al; Misdiagnosis and delay in referral of children with localized scleroderma. Br J Dermatol. 2011 Dec;165(6):1308-13. doi: 10.1111/j.1365-2133.2011.10600.x. Epub 2011 Nov 2.
- Morphoea; DermNet NZ
- Kreuter A; Localized scleroderma. Dermatol Ther. 2012 Mar-Apr;25(2):135-47. doi: 10.1111/j.1529-8019.2012.01479.x.
- Prinz JC, Kutasi Z, Weisenseel P, et al; "Borrelia-associated early-onset morphea": A particular type of scleroderma in childhood and adolescence with high titer antinuclear antibodies? Results of a cohort analysis and presentation of three cases. J Am Acad Dermatol. 2008 Nov 18.
- Alhathlool A, Hein R, Andres C, et al; Post-Irradiation Morphea: Case report and review of the literature. J Dermatol Case Rep. 2012 Sep 28;6(3):73-7. doi: 10.3315/jdcr.2012.1106.
- Maletic J, Tsirka V, Ioannides P, et al; Parry-Romberg Syndrome Associated with Localized Scleroderma. Case Rep Neurol. 2010 Jun 1;2(2):57-62.
- Zulian F, Cuffaro G, Sperotto F; Scleroderma in children: an update. Curr Opin Rheumatol. 2013 Sep;25(5):643-50. doi: 10.1097/BOR.0b013e3283641f61.
- Zwischenberger BA, Jacobe HT; A systematic review of morphea treatments and therapeutic algorithm. J Am Acad Dermatol. 2011 Nov;65(5):925-41. doi: 10.1016/j.jaad.2010.09.006. Epub 2011 Jun 8.
- Kerr AC, Ferguson J, Attili SK, et al; Ultraviolet A1 phototherapy: a British Photodermatology Group workshop report. Clin Exp Dermatol. 2012 Apr;37(3):219-26. doi: 10.1111/j.1365-2230.2011.04256.x. Epub 2012 Jan 25.
- Cantisani C, Miraglia E, Richetta AG, et al; Generalized morphea successfully treated with tacrolimus 0.1% ointment. J Drugs Dermatol. 2013 Jan;12(1):14-5.
- Piram M, McCuaig C, Saint-Cyr C, et al; Short And Long Term Outcome Of Linear Morphea In Children. Br J Dermatol. 2013 Sep 6. doi: 10.1111/bjd.12606.
- Saxton-Daniels S, Jacobe HT; An evaluation of long-term outcomes in adults with pediatric-onset morphea. Arch Dermatol. 2010 Sep;146(9):1044-5.
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