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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 nearly all cases the cause of morphoea is unknown. It can however sometimes follow:
- Localised injury
- Tick bites – it has been associated with Lyme disease due to Borrelia burgdorferi infection (not known in New Zealand)
- Pregnancy
- Measles and other viral infections
- Autoimmune diseases including lichen sclerosus and lichen planus.
Clinical features
- Plaques. This is the most common pattern of morphoea. There are 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.
- 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. A long and narrow plaque may be associated with underlying contractures.
- 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.
- Generalised morphoea. This is a very rare form of morphoea with widespread skin thickening over the trunk in this.
- Pansclerotic disabling morphoea. This affects children and results in extensive hardening of skin and underlying muscle. The growth of bones may be affected.
- Atrophoderma of Pierini and Pasini is thought to be a form of morphoea in which severe loss of subcutaneous tissue arises so that there is a depression within the skin.
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En coup de sabre |
Generalised morphoea |
Natural history
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 |
Treatment
Unfortunately there is no available effective treatment for most cases of morphoea.
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
- Diphenylhydantoin (phenytoin)
Related information
On DermNet NZ:
Other websites:
- Scleroderma – Medline Plus
- Scleroderma in children – from Paediatric Rheumatology site
- Raynaud and Scleroderma Association
- Morphea – emedicine dermatology, the online textbook