Linear Morphea: Linear scleroderma…

Linear Morphea is also known as Linear Scleroderma.  Scleroderma is characterized by the appearance of circumscribed or diffuse, hard, smooth, ivory-colored areas that are immobile and give the appearance of hidebound skin (Andrews’ Diseases of the Skin).  There are many forms, which Linear Morphea is one…which often develops in children, usually in the first decade of life.  In the inflammatory phase the lesions may appear pink, purple or bruise-like.  Once sclerosis sets in the skin becomes hard, thickened, bound down, shiny, ivory white and may have a purple border of surrounding inflammation.

These linear lesions often develop along an arm or leg and may follow lines of Blaschko, but may involve nearly any part of the body to include the face (resulting in hemifacial atropy of Parry-Romberg syndrome), as well as the trunk. En coup de sabre is a unique form involving the central facial regions along the forehead and will be reviewed separately.  When the lower extremity is involved there may be associated spina bifida, faulty limb development, hemiatrophy and/or flexion contractures.

Melorheostosis may be observed on radiographs as a dense, linear cortical hyperostosis.  At times linear lesions of the trunk merge into more generalized involvement.  Dyspigmentation and atrophy occur over many months to years.  Hyperpigmentation is more common than hypopigmentation and superficial dermal atrophy is manifested as shiny skin, often with visible blood vessels.  Deeper atrophy affecting fat, muscle and bone may occur.

ETIOLOGY:  Major advances have been made in the molecular studies of morphea and systemic sclerosis, however, their etiologies and pathogenesis remain a mystery…they both result in activation of similar inflammatory and fibrotic pathways.

TREATMENT:  Topical therapy may be helpful in some cases early on, to include topical steroids, tacrolimus ointment, calcipotriol ointment or cream and imiquimod.  Photothearpy can soften morphea and has some anti-inflammatory characteristics, but produces long-term complications with UV exposure.  Systemic treatment includes methotrexte, systemic corticosteroids and mycophenolate mofetil.  Physical therapy of the involved limb is of paramount importance to prevent contractures and frozen joints.  To read more click HERE and HERE.