Localised Scleroderma

There are many different types of localised scleroderma (also known as morphoea), which vary significantly in terms of their severity. It depends on how much of the skin is involved, whether any structures beneath the skin such as fat, fascia, muscle and bone are affected; and whether the condition is active and progressing.

Plaque morphoea:

• Limited plaque morphoea: localised patches of hardened skin that appear smooth and shiny. They usually appear on the trunk, but they can affect any part of the body. The condition is painless and there are normally no other symptoms or additional problems. This is the most common type in adults.

• Disseminated plaque morphoea: occurs at more than two anatomical sites and plaques may join together. The plaques may be isomorphic at sites of friction or non-isomorphic at any place on the body.

• Pansclerotic morphoea: begins on the trunk or limbs and spread quickly over a period of weeks or months. It can cause restriction of skin movement and difficulty with expansion of the chest when breathing and is more likely to affect deeper tissues. It is very rare, and most severe form of morphoea. There is an overlap between pansclerotic morphoea and eosinophilic fasciitis.

Sometimes, the term ‘generalised morphoea’ is used to include both disseminated plaque morphoea and pansclerotic morphoea. Since these sub-types are very different in terms of severity and the need for treatment, we avoid using the term ‘generalised morphoea,’ simply to avoid confusion.

Linear morphoea: 

• Linear Morphoea: the skin is affected in the form of a line, usually along an arm or a leg. The skin appears shiny, discoloured or scarred, and often feels tight and uncomfortable. In children, this should be monitored carefully because the ongoing growth of the limbs can be affected.

• En coup de sabre: literally means ‘cut of a sword’ and is when linear morphoea appears on the scalp, temple and neck. If the affected area is confined to the scalp then the problem is mainly cosmetic, although the underlying bone may be affected.

• Parry Romberg syndrome: when the linear morphoea presents on the face, and occasionally the tongue. This requires careful monitoring as it may affect the growth of the facial bones.

Whilst all subtypes described above can have superficial or deep involvement, the linear and pansclerotic forms are most likely to involve structures below the skin such as fat, fascia, muscle and bone. They should be monitored closely and are more likely to need treatment.