Pediatric morphea (scleroderma)

What is pediatric morphea (scleroderma)?

Morphea (mor·​phea) is an uncommon persistent condition in which there are areas of thickened skin. It is also known as localized scleroderma (sclero·​der·​ma). It may affect adults or children.

Clinical features of pediatric morphea (scleroderma)

• Plaques - This is the most common pattern of morphea. There are thickened scar-like oval patches of skin from 1- 20 cm or more in diameter. They start off purple or pink in color, then over several months they usually become ivory white in the middle with a purple 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. Over time, the affected area may appear sunken.

• Linear scleroderma - This is most often found on the limb of a child. A long and narrow or large plaque may be associated with tight, bound-down skin or restricted movement of the limb.

• En coup de sabre - This is a deep-seated form of linear scleroderma affecting the scalp and temple like from a sabre (sword). The hair is lost permanently and the underlying skull bone may shrink.

• Generalized morphea - This is a very rare form of morphea with widespread skin thickening over the body.

• Pansclerotic disabling morphea - 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

Natural history of pediatric morphea (scleroderma)

Plaque-type morphea is usually active for several years then slowly softens, leaving brown staining and sometimes depressed areas of skin. Linear morphea lasts longer, but also eventually improves, although sometimes deposits of calcium arise within the lesions. Limbs affected by severe morphea may be stiff and weak if there is muscle wasting.

What is the cause of pediatric morphea (scleroderma)?

In nearly all cases the cause of morphea is unknown. It can however sometimes follow:

• Localized injury

• Tick bites - it has been associated with Lyme disease

• Pregnancy

• Measles and other viral infections

• Autoimmune diseases including lichen sclerosus and lichen planus

How is pediatric morphea (scleroderma) treated?

• Topical calcipotriol

• Intralesional steroid injections

• Phototherapy

• Cyclosporin

• Pentoxifylline

• Penicillamine

• Diphenylhydantoin (phenytoin)

• Methotrexate

• Antibiotics