Are these progressive skin changes a sign of systemic disease?

Dermclinic
A Photo Quiz to Hone Dermatologic Skills

Case: This 17-year-old boy has had a thickening of his skin with hypopigmentation on the right side of his neck for the past 6 months. The area of thickening has slowly enlarged and is asymptomatic. The patient reports no limitation of neck movement. 

Are these progressive skin changes a sign of systemic disease? 
 

(Answer on next page.)

Dermclinic—Answer

No, morphea is a localized, self-limited skin disorder

Morphea is a slowly progressive fibrosing condition that affects the skin and subcutaneous tissue. Although morphea is sometimes called localized scleroderma, it is not related to scleroderma (systemic sclerosis). The histopathology of morphea and systemic sclerosis is identical; however, the 2 conditions are morphologically unique in their clinical presentation and prognosis.

Morphea is a rare skin disorder, which is generally classified according to its morphology: plaque (most common), generalized, linear, bullous, and deep. The more extensive or deep the morphea is and the more critical its location, the greater the associated morbidity. For instance, when morphea develops over a joint, sclerosis can progressively limit mobility; when the sclerosis involves the muscle underlying the affected skin, muscle atrophy can occur.

An individual plaque of morphea begins as a patch of red to violaceous skin that later becomes sclerotic and spreads centrifugally. The characteristic sclerotic plaque with white center and violaceous peripheral border appears when the inflammatory\sclerotic process is active. This process permanently alters the skin pigmentation and texture. The natural course of plaque disease is to “burn out” (soften and stop progressing) over a few years and leave postinflammatory changes.

This adolescent boy has the superficial variant of plaque morphea because the inflammatory\sclerotic process involves the epidermis and dermis. The treatment of plaque-type morphea is based on the extent, depth, and location of the sclerotic plaque. For useful treatment algorithms, see the 2-part article on morphea by Fett and Worth.^1,2 The algorithm for limited plaque morphea suggests using tacrolimus 0.1% twice daily for 8 weeks. If this treatment is not effective, then narrow-band UVB phototherapy and\or topical betamethasone dipropionate\calcitriol are second-line therapies. When the plaques are generalized or over joints, systemic corticosteroids and methotrexate are the treatments of choice.

The response to treatment is difficult to gauge clinically. The first change is the reduction and eradication of erythema, followed by softening of the skin texture. This teenage boy is being treated with tacrolimus 0.1% twice daily. He has yet to return for follow-up. ■

References:

1. Fett N, Worth VP. Update on morphea. Part I. Epidemiology, clinical presentation, and pathogenesis. J Am Acad Dermatol. 2011;64:217-228.
2. Fett N, Worth VP. Update on morphea. Part II. Outcome measures and treatment. J Am Acad Dermatol. 2011;64:231-242,