Morphoea

Morphoea (morphea) is an uncommon persistent condition in which there are areas of thickened skin. It is also known as localised scleroderma. It may affect adults or children.

In most cases the cause of morphoea is unknown. It can however sometimes occur with systemic sclerosis, or follow:¹

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

• Plaques are the most common pattern of morphoea. There are thickened, oval patches of skin between 1- 20 cm (or greater) in diameter. They start off mauve in colour, then over several months they usually become ivory white in the middle with a lilac edge. Old lesions may be brown. The surface is smooth, shiny and hairless. The patches do not sweat. Several plaques may be present, on both sides of the trunk and limbs distributed asymmetrically. Sometimes the surface is just hyperpigmented with very little to feel.
• Superficial morphoea usually presents in middle-aged women as symmetrical mauve-coloured patches in the skin folds. They are found in the groin, armpits and under the breasts.
• On the scalp/forehead and on the limbs morphoea may be linear. 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 affects children and results in extensive hardening of skin and underlying muscle. The growth of bones may be affected.
• More rarely there is generalised morphoea, with widespread skin thickening over the trunk.

Blood tests have little role in assessment of morphoea.
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.²

• For plaque-type and generalized 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.

• 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 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.²Treatment of active lesions with superpotent topical or intralesional corticosteroids may help reduce inflammation and prevent progression.

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.³
Occasionally the following are found helpful:

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

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.