Morphoea (US spelling is morphea) is an uncommon persistent condition in which there are areas of thickened skin, and occasionally underlying tissues including bone. It is also known as localised scleroderma. It may affect adults or children, and is at least twice as common in females as males. It is more common in people with white skin compared to darker skin types.
It is important to distinguish morphoea from systemic sclerosis, a multiorgan systemic disease in which there is also hardening of the skin. Unlike systemic sclerosis, morphoea does not result in spindled fingers (sclerodactyly), Raynaud phenomenon or nailfold capillary changes.
What is the cause of morphoea?
In nearly all cases the cause of morphoea is unknown and is presumed to have both genetic and environmental components. Morphoea is associated with increased collagen and extracellular matrix in the dermis but it is not clear how this occurs. It appears to start off with damage to small blood vessels and the release of cytokines (chemical messenger proteins) that promote fibrosis (scarring).
Morphoea can sometimes follow:
- Localised injury, including injections and radiotherapy
- Tick bites – it has been associated with Lyme disease due to Borrelia burgdorferi infection (not known in New Zealand)
- Measles and other viral infections
- Autoimmune diseases including vitiligo, diabetes, thyroid disease, lichen sclerosus and lichen planus
- Medications including bleomycin and penicillamine.
Clinical features of morphoea
Morphoea is classified into several different types according to the appearance of the skin lesions. Several types may be present in one patient (mixed variant).
|Circumscribed or plaque morphoea||This is the most common pattern of morphoea. There are 1 to 3 thickened scar-like oval patches of skin from 1- 20 cm or more 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 and most often on breasts or hips. It may involve all layers of the skin and/or underlying tissues.|
|Generalised morphoea||This describes 4 or more plaques of morphoea affecting 2 or more body regions.|
|Superficial Morphoea||Usually affecting middle-aged women, superficial morphoea presents as symmetrical mauve-coloured patches in the skin folds, particularly the groin, armpits and under the breasts.|
|Linear scleroderma||This is most often found on the limb of a child, and is bilateral in some cases. A long and narrow plaque may be associated with underlying contractures. It may follow the lines of Blaschko, possibly due to cutaneous mosaicism.|
|En coup de sabre||This is a deep-seated form of linear scleroderma affecting the scalp and temple like a sabre cut. The hair is lost permanently and the underlying skull bone may shrink. Rarely, the central nervous system is affected.|
|Progressive facial hemiatrophy||Also called Parry-Romberg syndrome, there is loss of tissue under the skin (fat, muscle and bone) on one side of the face.|
|Pansclerotic disabling morphoea||This 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, nonhealing ulcers and squamous cell carcinoma. It is very rare.|
|Atrophoderma of Pierini and Pasini||A form of morphoea in which severe loss of subcutaneous tissue arises so that there is a depression within the skin.|
En coup de sabre
Some children and adults with morphoea complain of muscle and joint pains as well as fatigue. Seizures, headaches, vascular malformation, ocular defects and other neurological symptoms may be associated with morphoea, especially in those with en coup de sabre or progressive facial hemiatrophy.
There is an increased incidence of autoimmune disorders in the family members of children and adults with morphoea, but a family history of morphoea is rare (<1%).
Natural history of morphoea
Plaque-type morphoea is usually active for several years then slowly softens, leaving brown staining and sometimes depressed areas of skin. Linear morphoea 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.
Pigmentation from burnt-out morphoea
Subcutaneous atrophy due to morphoea
Should any tests be performed?
When morphoea is clinically typical, tests may be unnecessary. However, the following tests may be recommended in some cases.
- Skin biopsy to confirm the presence of scleroderma.
- Imaging (ultrasound, X-rays and MRI) to determine the extent of the deformities.
- Autoantibodies including antinuclear factor (commonly present in low to moderate titre). Antitopoisomerase II alpha antibody, if available, may be detected in most morphoea patients.
What is the treatment for morphoea?
The best treatment for morphoea is uncertain and current treatments often fail to help.
Benefit has been reported in some cases from the following treatments.
- Topical calcipotriol
- Topical tacrolimus
- Potent topical steroids
- Imiquimod cream
- Photochemotherapy or phototherapy with NBUVB or UVA1
- Systemic steroids
- Diphenylhydantoin (phenytoin)
Physiotherapy is often recommended to prevent joint contractures when morphoea affects the limbs.