A 57-year-old woman presented with a 2-year history of thickened skin lesions over the dorsum of the left hand. There was significant functional impairment with restriction in active range of movement. She has a known history of rheumatoid arthritis, Grave’s disease and Conn’s syndrome. Physical examination revealed linear thickened and hypo-pigmented lesions on the left index and middle fingers. Other body parts were uninvolved. There was no microstomia, telangiectasia, sclerodactyly, calcinosis cutis or digital vasculitic changes.
Skin biopsy showed swollen collagen bundles throughout the thickened dermis. Adnexal structures were atrophic. A predominantly perivascular plasma cell-rich inflammatory cell infiltrate was noted. The histological feature was compatible with the diagnosis of morphea.
Morphea is a form of localized scleroderma, with predominant skin involvement. Localized scleroderma differs from systemic sclerosis based on the clinical findings of almost exclusive cutaneous lesions with absence of internal organ involvement. Localized scleroderma is sub-classified into
- Generalized morphea and
- Linear scleroderma (morphea en coup de sabre)
Morphea presents in the form of one or several indurated plaques. Active lesions are surrounded by a violaceous border whilst established lesions may be hyper- or hypo-pigmented. Linear scleroderma represents a special variant with linear ivory-like depressed lesions over the face and resembles a classic saber bow. Anti-nuclear (ANA), anti-topoisomerase-I (Scl-70) and anti-centromere antibodies are often present.
Histologically, there are thickened collagen bundles within the reticular dermis with a moderately severe inflammatory cell infiltrate, predominately lymphoplasmacytic between collagen bundles and around blood vessels. The infiltrate may extend into the subcutaneous fat. Collagen may replace fat cells in the subcutaneous tissue at the late stage.
Therapy in morphea has been challenging. Successful treatment has been reported with systemic steroids, methotrexate, d-penicillamine, calcipotriol and phototherapy. Non-pharmacological measures such as physiotherapy may help in enhancing dermal elasticity and functional capacity.