Morphea (aka Localized Scleroderma)

Morphea, also known as localized scleroderma, is an inflammatory skin disease that affects the dermis and subcutaneous fat and leads to a scar-like sclerosis.
Morphea can present in a patchy distribution or as a linear plaque.
Morphea can occasionally extend deep to the underlying fascia, muscle, or bone.
Unlike systemic sclerosis, patients with morphea do not have involvement of internal organs or Raynaud phenomenon.
The female-to-male ratio is 3:1.

Clinical Manifestations ⬆ ⬇

Morphea can be seen as a single plaque, multiple plaques in a widespread distribution (generalized morphea), or a single linear plaque.
An individual lesion initially appears as a localized, pink-red to violaceous slightly raised plaque. Subsequently, the plaque becomes an indurated, hairless lesion with a characteristic “active” “lilac” border that represents disease expansion (Fig. 34.31).
As plaques expand the central part becomes white and scar-like and progressively indurated.
Lesions are commonly found on the trunk or extremities or they may become widespread (generalized morphea).
Linear morphea usually begins as a single plaque that then expands longitudinally most often on an extremity.
Occasionally, linear morphea can severely affect the mobility of the affected extremity, especially if the condition crosses a joint.
Morphea is generally asymptomatic, but itching may occur in the initial stages
Morphea usually “burns out” spontaneously, and leaves post-inflammatory hyperpigmentation or a scar.

Clinical Variant ⬆ ⬇

Linear morphea of the head is referred to as “morphea en coup de sabre” and is usually located on the lateral forehead and extends into the frontal scalp.
The affected area is likened to a cut from a saber (Fig. 34.32).
En coup de sabre morphea can involve the underlying muscles and bones or rarely, the meninges and even the brain.
Parry-Romberg syndrome, also called hemifacial atrophy, is considered a very severe variant of linear morphea seen on the face in the distribution of the trigeminal nerve.
Deep, guttate, or nodular forms are less common variants.

Diagnosis ⬆

Differential Diagnosis

Lichen Sclerosis (also Known as Lichen Sclerosis et Atrophicus [LSA])

Atrophic isolated plaque(s) that resembles morphea.
May coexist with morphea.
Lesions consist of white plaques with epidermal atrophy and “cigarette paper” wrinkling.
May involve genitals.

Management

Topical, intralesional, and systemic steroids may be helpful in the early inflammatory stage.
Topical vitamin D analogs (calcitriol, calcipotriene) are sometimes effective.
Treatment with UVB, UVA, and methotrexate may also be of some benefit.
For more extensive cases and linear morphea that crosses a joint systemic corticosteroids and methotrexate are often used to halt progression of the disease.

Helpful Hints

Some evidence suggests that some European cases of morphea (Lyme disease) may result from Borrelia burgdorferi infection. This connection has not been demonstrated in the United States.
Resolution of the advancing lilac border is a clinical indication that the morphea is no longer active.