- A 10-year-old girl presents to your office with a lesion diagnosed 4 years ago as a nevus simplex (salmon patch) on her back. Recently, the lesion has enlarged, darkened, and developed an area that is hard and white
- Of the following, the BEST diagnosis based on the clinical findings is
A. lichen scleroses
B. localized scleroderma
C. neurofibromatosis
D. tinea versicolor
E. vitiligo - The child described in the vignette has localized scleroderma (LS). Localized scleroderma has several subtypes including plaque morphea, generalized morphea, bullous morphea, linear morphea, and deep morphea—that are characterized by the pattern and depth of skin involvement. Early lesions of LS are characterized by a lilac ring or violaceous inflammatory border. The skin is indurated throughout the lesion. The lesions may enlarge and new lesions may occur. Because of uncontrolled inflammation, tissue damage accumulates as the diseases progresses, causing skin thickening and, commonly, an ivory-colored center of sclerosis (Item C62A). Dermal and subcutaneous atrophy can develop later in the disease course, resulting in visible veins, a flat or concave “cliff-drop” appearance to the skin (depression of the underlying subcutaneous tissues), and a lack of hair growth. Postinflammatory hyperpigmentation or hypopigmentation often occurs. The changes are often described as a “bruise that does not heal.”
- OLinear scleroderma is a subtype of scleroderma that presents as a single linear band that affects the head, trunk, or extremities. Linear scleroderma is usually unilateral. If the linear band of scleroderma crosses joints, it can affect the underlying structures, including muscle, tendon, joint capsule, and growth plate. These lesions can result in atrophy or shortening of the limb and joint contractures. Both linear scleroderma affecting the scalp and forehead (also called en coup de sabre) and Parry-Romberg syndrome (a form that causes hemifacial atrophy) can involve the central nervous system (CNS). Affected individuals are at risk for seizures, chronic headache, and optic neuritis and should undergo cranial magnetic resonance imaging (MRI) if CNS symptoms are present. Findings on MRI can include calcifications, white matter changes, vascular malformations, and signs of CNS vasculitis. Several other subtypes of LS exist, varying from single lesions (morphea) to more generalized involvement of the limbs (pansclerotic morphea). Untreated LS can result in growth abnormalities, disfiguring lesions, and joint contractures.
- Diagnosis of LS can be made by clinical examination or skin biopsy. Treatment for small isolated lesions can be topical with phototherapy, topical steroids, or other topical immunomodulating agents. Large or multiple lesions, especially those involving joints or the face, are often treated systemically to prevent permanent damage and disfigurement.
- No standardized treatments exist; however, most rheumatologists use a combination of steroids and methotrexate to control the disease. Expectations for improvement in the lesions are dependent on the stage of disease at the time of treatment. Once sclerosis and scarring have occurred, very little improvement can be expected. Referral to dermatology or rheumatology in order to improve outcomes is warranted for these patients.
- Lichen sclerosus lesions are scaly and typically hypopigmented, with a cigarette paper-like wrinkled appearance and varying degrees of sclerosis . Lichen sclerosis can be present concurrently with morphea. Neurofibromatosis can present with ash leaf spots but without evidence of induration. Tinea versicolor presents as erythematous, hypopigmented, or hyperpigmented macules that are most commonly over the trunk andupper extremities but without skin thickening . Vitiligo presents with depigmented macules and patches without skin thickening . Localized scleroderma, note the ivory-colored central area
Dr. Jayaprakash
Superintendent, ICH.
Institute of Child Health.
Gov. Medical College Kottayam.
Kerala, India.