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Morphea_0213 Derm Clinic
A girl, aged 11 years, presented to the dermatology clinic with complaints of a slowly spreading discoloration on her right arm. The discoloration started six months earlier as a small dark patch on her dorsal wrist and spread upward toward the elbow. She was previously treated for a presumed fungal infection with a topical antifungal and a medium-potency topical steroid.
Physical examination revealed smooth, shiny, and firm hyperpigmented patches on the dorsal wrist, forearm, and elbow. Mobility of the local joints was normal. The girl had no significant medical history and was taking no medication.
HOW TO TAKE THE POST-TEST: This Clinical Advisor CME activity consists of 3 articles. To obtain credit, you must also read Red scalp plaque with painful ulceration and Intertriginous erosions.
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A 4-mm punch biopsy was ordered and was consistent with morphea, a form of localized cutaneous scleroderma. Scleroderma, an autoimmune connective-tissue disorder, presents in localized and systemic forms. When the disease is localized to the skin and underlying connective tissue only, it is referred to as morphea or localized scleroderma.
Morphea appears with atrophic ivory or dyspigmented patches and plaques, most commonly on the trunk. The affected area appears firm or sclerotic, and local skin elasticity is greatly diminished.
Plaque-type morphea, the most common presentation in adults and the second most common presentation in children, begins with erythematous or ivory patches that evolve into a hyperpigmented or ivory induration over the course of weeks to months.
A conspicuous violaceous peripheral border is often present. The size of the plaques ranges from a few centimeters to several inches. Guttate morphea, a subtype of plaque morphea, presents with multiple scattered 1-mm to 3-mm superficial papules on the trunk and/or extremities.
Linear morphea is the most common presentation in children and presents with linear bands of induration and either hyperpigmentation or hypopigmentation. Linear morphea that involves the frontal scalp is termed en coup de sabre and may cause neurologic complications.
Parry-Romberg syndrome represents a more severe form of linear facial morphea, with progressive atrophy of half the face. Linear scleroderma that involves an extremity in a pediatric patient may cause stunted growth of the limb and possibly joint contractures. When linear scleroderma involves the chest and breast area in prepubertal females, it can cause underdevelopment of the breasts.
Generalized morphea presents with widespread tight indurated plaques that may be pink, ivory, or hyperpigmented. Muscle atrophy may be present. Both pansclerotic morphea and morphea profunda have deeper involvement, including the dermis, panniculus, fascia, muscle, or even bone.
Localized scleroderma has been reported to have an incidence of 0.4 to 1.0 per 100,000. The localized form is 10 times more common than the systemic form in children. Whites and females are affected at a significantly higher rate.
The pathogenesis of scleroderma is multifactorial. Both vascular damage and autoimmune dysfunction are thought to be involved. Environmental triggers and trauma may play a role in genetically susceptible patients. An association between morphea and Borrelia burgdorferi infection has been investigated,1 but no clear conclusion has been presented.
Although the diagnosis of morphea is often made clinically, lesional skin biopsy is necessary in questionable cases. Histologic examination of morphea shows an inflammatory cell infiltrate and thickened collagen bundles within the dermis at earlier stages, and sclerosis, atrophy, and closely packed collagen later in the course of the disease.
Treatment for cutaneous scleroderma is challenging. Some individuals, particularly children, may experience spontaneous improvement within three to five years when the disease is localized.2 However, the pigment change—and occasionally the atrophy—may persist.
Treatment with topical calcipotriene (Dovonex) early in the course of the disease may halt progress. Potent topical steroids may hasten resolution of the lesions, but the risk of localized atrophy must be considered.
More severe disease requires systemic treatment. Low-dose methotrexate (Rheumatrex, Trexall) in combination with pulsed high-dose systemic steroids offers very promising results and is often considered first-line therapy for severe disease.3 Other options include oral mycophenolate mofetil (CellCept), oral calcitriol (Rocaltrol), and psoralen plus UVA light therapy (PUVA).
Tightening of the skin may lead to impaired function of the affected area. Cosmetic appearance is often a concern.
The patient in this case was treated with clobetasol ointment applied in the mornings and calcipotriene foam (Sorilux) applied in the evenings. Six months later, the disease was stable, and the patient had noticed no worsening of the discoloration or skin texture.
Esther Stern, NP, is a family nurse practitioner at Advanced Dermatology & Skin Surgery, P.C., in Lakewood, N.J.
References
1. Prinz JC, Kutasi Z, Weisenseel P, et al. “Borrelia-associated early-onset morphea”: a particular type of scleroderma in childhood and adolescence with high titer antinuclear antibodies? Results of a cohort analysis and presentation of three cases. J Am Acad Dermatol. 2009;60:248-255.
2. Paller AS, Mancini AJ. Clinical Pediatric Dermatology: A Textbook of Skin Disorders of Childhood and Adolescence. 4th edition. Philadelphia, Pa.: Elsevier Saunders; 2011:522.
3. Kreuter A, Gambichler T, Breuckmann F, et al. Pulsed high-dose corticosteroids combined with low-dose methotrexate in severe localized scleroderma. Arch Dermatol. 2005;141:847-852.
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