Morphea and Lichen Sclerosus

Published on 05/03/2015 by admin

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Last modified 05/03/2015

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Morphea and Lichen Sclerosus

Morphea (Localized Scleroderma)

An uncommon fibrosing disorder that is limited to the skin, subcutaneous tissues, and occasionally the underlying bone; rarely, if present on the face and/or scalp, it can be associated with underlying CNS abnormalities.

It is distinct from systemic sclerosis (SSc; see Chapter 35) in that morphea is not associated with sclerodactyly, Raynaud’s phenomenon, nailfold capillary abnormalities, or internal organ involvement.

Morphea does not transition into SSc, except for a few rare case reports.

Equal prevalence in adults and children; more common in females and Caucasians.

Pathogenesis unknown but thought to involve (like SSc) vascular damage, immune activation, and increased connective tissue production by fibroblasts.

Approximately 2–5% of children and 30% of adults with morphea have a concomitant autoimmune disease (e.g. alopecia areata, vitiligo) as well as a family history of autoimmunity.

Classified based on clinical presentation, with five major variants recognized (see Fig. 35.1; Table 36.1).

Early morphea lesions present as erythematous to violaceous patches and plaques (Fig. 36.1); they then evolve into sclerotic (often ivory-colored), hairless, anhidrotic plaques with variable degrees of dyspigmentation (Fig. 36.2).

Circumscribed (plaque) morphea is the most common variant in adults, presenting with ≤3 discrete indurated plaques; the latter favor the trunk and tend to develop in areas of pressure (e.g. hips, waist, and bra line in women); superficial and deep variants (morphea profunda) exist (Fig. 36.3).

Generalized morphea is a rare variant that presents with >3 indurated plaques larger than 3 cm and/or involving ≥2 body sites; spares the face and hands (see Fig. 36.11); more likely to have a (+) ANA and systemic symptoms.

Linear morphea is the most common variant in children and may cause a significant degree of morbidity because of ocular involvement and occasionally CNS involvement in the head variant (Fig. 36.4) or muscle atrophy, discrepancies in limb length, and joint contractures in the limb variant (Fig. 36.5).

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