Early recognition and treatment of scarring skin disorders: Focus on acne vulgaris and morphea
by Yvonne Chiu, MD
Yvonne Chiu, MD, is a pediatric dermatologist at Children's Hospital of Wisconsin, an assistant professor of Dermatology at The Medical College of Wisconsin and a member of Children's Specialty Group.
|Children's Hospital of Wisconsin has one of the largest pediatric dermatology practices in the country with about 15,000 patient visits each year. Yvonne Chiu, MD, and her colleagues treat a wide range of conditions in the following clinics: Atopic Dermatitis, General Dermatology, Birthmarks and Vascular Anomalies, Hemangiomas of Infancy, Hyperhidrosis, Laser, Surgery and Dermatology-Oncology.|
In contrast to acne, morphea is a rare skin disease with yearly incidence rates of 25 per million and lifetime prevalence rates of 2,000 per million. Although rare, morphea is invariably scarring, and 10 percent of affected patients develop functional disability as a result of the scars. Because of its rare nature and insidious onset, morphea usually is not recognized immediately and substantial scarring already may have occurred by the time of referral.
Regrettably, the treatment of skin scarring is limited, often expensive and will improve but not reverse the scar. The most important management option is early recognition and treatment, before further scarring occurs. In general, any child with a scarring skin disorder should be referred in a timely manner to a pediatric dermatologist for management. (See Table 1.)
Acne vulgaris encompasses a wide range of clinical features. Open comedones (whiteheads) and closed comedones (blackheads) form when keratinocytes and sebum accumulate within a hair follicle. Comedones may progress to inflammatory lesions when the commensal bacteria Propionibacterium acnes proliferates and the comedo ruptures. Inflammatory acne lesions may be relatively small and superficial, 1-5 mm, erythematous papules and pustules. Severe inflammatory disease is characterized by large, deep, tender nodules and cysts.
Scarring from acne takes many forms. Most patients should be reassured that postinflammatory hyperpigmentation and persistent erythema are not permanent and slowly will resolve. Icepick scarring may result from superficial papules and pustules, and extremely rarely from comedones. Pitted scars typically are caused by nodulocystic lesions and are deeper and wider. Hypertrophic and nodular scars may develop from severe disease on the trunk. In addition to physical scarring, acne vulgaris and its subsequent scarring can have important effects on quality of life and self-image.
Comedonal and mild papulopustular acne vulgaris typically are treated with topical medications alone. More severe papulopustular and nodulocystic disease require treatment with systemic agents. Oral antibiotics, typically of the tetracycline class, have anti-Propionibacterium acnes and anti-inflammatory effects. Hormonal therapy such as estrogen-containing oral contraceptives and spironolactone may be helpful for female patients. Isotretinoin is the gold-standard for severe nodulocystic disease that is refractory to other therapies and also may be beneficial for less severe but scarring disease. Isotretinoin results in complete clearance of acne in 85 to 90 percent of treated patients, and in contrast to other acne therapies that require continued treatment to maintain clearance, isotretinoin can induce a lasting remission of acne vulgaris. The typical dosing is 0.5-1 mg/kg/day over a 4-6 month course for a cumulative dose of 120-150 mg/kg. Isotretinoin has numerous side effects, including teratogenicity, and it is best managed by experienced practitioners. In an attempt to reduce fetal exposure to isotretinoin, the U.S. Food and Drug Administration has instituted a mandatory iPLEDGE registry for all prescribers, patients, wholesalers and pharmacies.
Morphea, also known as localized scleroderma, is a rare connective tissue disease characterized by sclerosis of the skin and subcutaneous tissues. Children comprise almost half of all cases, and girls are more often affected than boys. Lesions typically start with the insidious onset of firm, violaceous or erythematous plaques. The plaques gradually enlarge with active, violaceous or erythematous borders and shiny, white, firm centers. Atrophic, sclerotic and depressed scars are the eventual sequelae. Progression to systemic sclerosis is exceedingly rare. As morphea evolves very slowly, parents and care providers may not notice the disease initially.
There are multiple types of morphea, with the linear form most common in children. Plaque morphea manifests as single or multiple discrete lesions, primarily on the trunk and ranging in size from 1-20 cm. Linear morphea most often occurs longitudinally along an extremity. As linear morphea can cause deeper sclerosis of subcutaneous tissue, muscle and bone, erythema and other signs of active inflammation in the skin may not be obvious. Flexion contractures or scoliosis are potential complications. Variants of linear morphea can occur on the frontal scalp and forehead (en coup de sabre) or cause atrophy of half of the face (Parry-Romberg syndrome). (See Figure 1.) Both en coup de sabre and Parry-Romberg syndrome may be associated with abnormalities on MRI or CT and neurologic symptoms such as headaches and seizures. Other less common presentations of morphea include guttate morphea, generalized morphea, bullous morphea, disabling pansclerotic morphea and eosinophilic fasciitis.
Treatment for limited plaque morphea consists of topical corticosteroids, topical vitamin D analogues and topical tacrolimus or pimecrolimus. Extensive plaque morphea and most cases of linear morphea are treated with systemic agents. Generally, long-term methotrexate is administered with an initial course of oral prednisone or pulse intravenous methylprednisolone. Therapy is used to halt disease progression but does not reverse existing scarring, emphasizing the importance of early treatment.
Many skin conditions, both common and rare, may result in scarring. Acne vulgaris is commonly seen in adolescents but encompasses a wide spectrum of disease ranging from mild to severe. Early referral and treatment should be considered for any patient with scarring acne. Morphea is a rare and insidious connective tissue disease, and thus, it often is unrecognized. Timely diagnosis and therapy prevents functional complications and disfiguring scars.
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