Synonyms: localised scleroderma, morphea

Epidemiology¹

Morphoea is a rare, persistent condition where the patient has areas of thickened skin. Studies in the USA report an incidence of 25 cases per million population per year but the actual figure is likely to be far higher because many cases may not come to medical attention.

Aetiology¹

In most cases the cause of morphoea is unknown. One study found that 0.9-5.7% of patients with morphea progress to systemic scleroderma.

It has been known to follow:²

• Tick bites - it has been associated with Lyme disease.³
• Measles and other viral infections.
• Localised injury.
• Pregnancy.
• Autoimmune diseases including lichen sclerosus and lichen planus.
• Radiotherapy.

A few familial cases have been reported

Presentation

• Plaques:
  • 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 morphea:
  • 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 affects children and results in extensive hardening of skin and underlying muscle.
  • The growth of bones may be affected.
• Generalised morphoea:
  • This a rare condition with widespread skin thickening over the trunk.
• Extracutaneous manifestations:
  • These can include arthralgia, dysphagia and seizures (in patients with craniofacial linear morphoea).

Parry-Romberg syndrome is a rare neurocutaneous disorder in which linear scleroderma is associated with progressive facial hemiatrophy, migraine, facial pain and epilepsy.⁴

Investigations¹

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 (e.g. 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:¹

• 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.

Differential diagnosis

• 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.

Management¹

All suspected cases should be referred for diagnosis.

Unfortunately there is no available, effective treatment for most cases of morphoea. Although several have shown 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.¹ Treatment of active lesions with super potent topical or intralesional corticosteroids may help to reduce inflammation and prevent progression. Topical tacrolimus has also been successful with mild-to-moderate lesions.⁵

Therapy with topical calcipotriene may also be beneficial. Night occlusion, e.g. with plastic wrap, is used to increase penetration of the medication.

Patients with potentially disabling generalised, linear or deep morphoea typically require more aggressive therapy.

Occasionally, the following are found helpful:

• Topical calcipotriol.
• Imiquod.
• Methotrexate.
• Mycophenolate mofetil.
• Oral retinoids.
• Systemic steroids.
• Intralesional steroid injections.
• Photochemotherapy or phototherapy with UVA1.
• Long courses of oral penicillin or tetracycline antibiotics.
• Ciclosporin.
• Colchicine.
• Pentoxifylline.
• Penicillamine.
• Phenytoin.

Prognosis¹

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.
• Limbs affected by severe morphoea may be stiff and weak if there is muscle wasting.

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.⁶

Document references

1. Nguyen JV et al; Morphoea, Medscape, Jan 2010
2. Morphoea, DermNet NZ, February 2011
3. 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. [abstract]
4. 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. [abstract]
5. Stefanaki C, Stefanaki K, Kontochristopoulos G, et al; Topical tacrolimus 0.1% ointment in the treatment of localized scleroderma. An open label clinical and histological study. J Dermatol. 2008 Nov;35(11):712-8. [abstract]
6. 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.

Internet and further reading

• 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. [abstract]
• Tomiyoshi C, Wojcik AS, Vencato EM, et al; Case for diagnosis. Localized scleroderma or morphea. An Bras Dermatol. 2010 Jun;85(3):397-9. [abstract]
• Valanciene G, Jasaitiene D, Valiukeviciene S; Pathogenesis and treatment modalities of localized scleroderma. Medicina (Kaunas). 2010;46(10):649-56. [abstract]