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Morphea

Synonym:

Localized scleroderma

Morphea is a localized sclerosis of the skin of unknown etiology. There is increasing evidence that at least some cases are secondary to a Borrelia infection.
Early lesions typically show evidence of inflammation. A white firm plaque appears at the inflammatory site, surrounding by remaining inflammation. This plaque, over time, spreads peripherally. Eventually, the plaque may become depressed and telangiectatic vessels may be seen.
Hyperpigmentation can be present.
Localized lesions are typical in childhood with more generalized cutaneous forms more commonly seen in older patients.

Clinical variants:

  • Guttate morphea, also known as "white-spot disease". Guttate morphea consists of multiple lesions typically less than 1 cm in diameter.
  • Linear morphea. Usually unilateral, linear areas of morphea can develop particularly on the extremities.
  • En coup de sabre morphea resembles a sabre-cut, and is linear morphea of the forehead and scalp. The depressed white plaque develops with complete hair loss. There may be associated telangiectasia and hyperpigmentation. There may be corresponding atrophy of the side of the face and tongue. The bones of the skull underlying the lesion can also show changes.

Treatment:

  • Topical potent steroids such as clobetasol 0.05% bid and occasionally milder steroids, such as betamethasone 0.1% tid can be effective in localized lesions.
  • Intralesional triamcinolone diacetate 5-10 mg/ml injected into thicker plaques can also be helpful.
  • Systemic treatment is controversial and includes diphenylhydantoin, griseofulvin, and potassium para-aminobenzoate.
  • Generalized cutaneous morphea may respond to oral corticosteroids, but as treatment will be prolonged, care should be taken.
  • In view of the growing evidence of a Borrelia association, a trial of therapy with penicillin G may be appropriate in progressive disease.
morphea


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