Abstract

Morphea, also known as Localized scleroderma, is an idiopathic often self-limited, inflammatory disorder that causes fibrotic changes in the skin. Fibrotic, asymmetric plaques, usually 2-15 cm wide, characterize the disease. The active lesions may have erythematous or violaceous borders, whereas the inactive ones may result to hyperpigmentation. The thickening may expand to the subcutaneous tissue or may be to the lower layers of the skin, causing dysfunction at certain levels. There is no relevance to any systematic disease. Morphea usually evolves for several years and then degrades.

Highlights

  • Morphea or Localized Scleroderma is a disorder characterized by excessive deposition of collagen, resulting in thickening of the skin, the subcutaneous tissue, or both [1,2,3,4]

  • The incidence of the disease is 2.7/100,000 people in the general population, and the prevalence of the disease increases with age [11]

  • The male to female ratio is 1: 3, apart from the linear form, where the disease affects both sexes [12,13]. It occurs more frequently in Caucasians and Asians compared to African-Americans

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Summary

Introduction

Morphea or Localized Scleroderma is a disorder characterized by excessive deposition of collagen, resulting in thickening of the skin, the subcutaneous tissue, or both [1,2,3,4]. The overproduction of collagen by fibroblasts in the affected tissues is a common feature of all types of scleroderma, the fibroblast activation mechanism is still unknown [17,18]. The endothelial cells’ injury is believed to trigger the disease pathogenesis This results in the creation of adhesion molecules and T-helper 2 cytokines, such as interleukins IL-4 and IL-6, and transforming growth factor-beta [TGF-β]. Cytokines along with growth factors increase fibroblastic activity and induce further collagen synthesis and connective tissue [23]. Other possible causes of the disease onset include post-trauma inflammation, vaccination with BCG, parenteral administration of vitamin B, radiotherapy, chimerism, certain drugs [penicillamine, bromocriptine, etc.] [9,30]

Clinical Features
Extracutaneous Manifestations
Differential Diagnosis
Prognosis and Clinical Course
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