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INTRODUCTION

Morphea is localized scleroderma confined to the skin. It most commonly affects the trunk but also occurs on the face and extremities. The four clinical variants include plaque-type morphea, generalized morphea, linear morphea (en coup de sabre), and pansclerotic morphea of children (morphea profunda).

EPIDEMIOLOGY

Incidence: rare

Age: most commonly occurs in the second to fifth decade. Linear scleroderma and morphea profunda are more common in children

Race: slightly more common in Caucasians

Sex: females more than males (2–3:1)

Precipitating factors: Borrelia can trigger morphea in some cases, predominantly in Europe

PATHOGENESIS

Overproduction of collagen (types I, II, III) and glycosaminoglycans by skin fibroblasts and vascular damage. Probable T-cell mediated phenomenon.

PHYSICAL EXAMINATION

Ill-defined pink to violaceous, indurated 2- to 15-cm plaques that transform to smooth sclerotic ivory-colored plaques with a light violaceous border and a shiny surface. Postinflammatory hyperpigmentation is prevalent (Fig. 55.1). Linear morphea presents with a linear erythematous inflammatory streak that may progress to form a scar-like band involving underlying fascia, muscle, and tendons.

Figure 55.1

(A) Early morphea on the left leg presenting as an erythematous plaque. (B) Same patient with late stage morphea on the right leg presenting as linear depressed yellowish to white hard plaques with erythematous margins

DIFFERENTIAL DIAGNOSES

Acrodermatitis chronica atrophicans, eosinophilic fasciitis, lichen sclerosus et atrophicus, scleredema, scleromyxedema, and nephrogenic systemic fibrosis.

LABORATORY DATA

Serology

Check for Borrelia antibodies.

Dermatopathology

Homogenization and thickening of dermal collagen bundles, trapped and atrophic eccrine glands, perivascular mononuclear infiltrate of lymphocytes and plasma cells with normal or atrophic overlying epidermis. Underlying subcutaneous fat may also be involved with sclerosis in advanced cases.

COURSE

Course is variable. Many patients remit spontaneously but others have a progressive course.

MANAGEMENT

Treatment for this condition can be frustrating due to frequent treatment failure. Patients should be counseled that therapy may not be effective.

  • Topical treatment

    • – Corticosteroids

    • – Calcipotriene

  • Systemic treatment

    • – Corticosteroids, D-penicillamine, vitamin D3, methotrexate

  • Light treatment

    • – Ultraviolet A1 phototherapy

    • – Pulsed dye laser (585 nm, 5 J/cm2 twice monthly), reported to be effective in single case report

  • Subcision: subcision with a Nokor 18G needle may help to elevate the bound-down skin. It is most effective for linear morphea and facial hemiatrophy. Subcision is performed under local infiltrative anesthesia to the affected site with 1% lidocaine with 1:100,000 epinephrine. The Nokor needle is introduced at a 45-degree angle into the skin utilizing ...

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