Pediatric Morphea

Abstract

This article outlines the epidemiology, pathogenesis, clinical presentation, diagnosis, and treatment of childhood morphea. Also known as localized scleroderma, morphea is a fibrosing disorder of the skin and subcutaneous tissues. Morphea is differentiated from systemic sclerosis (scleroderma) based on the absence of sclerodactyly, Raynaud phenomenon, and nail-fold capillary changes. Confusion may occur because patients with morphea often have systemic symptoms such as malaise, fatigue, arthralgias, myalgias, and positive autoantibodies. Unlike morphea, systemic sclerosis has organ involvement, particularly gastrointestinal, pulmonary, and renal.

Systemic involvement in localized scleroderma/morphea

“…30 This type of LoSc most frequently involves extremities, causing not only muscle atrophy and severe cosmetic disfiguration but also limb length discrepancies, asymmetric extremity growth, and joint contractures. 30,31 The extremities may be thinner and shortened. 32 Another orthopedic finding in children is scoliosis, determined only in one study cohort in 5% of patients.…”

“…Even the generalized subset is differentiated from systemic scleroderma by the absence of this phenomenon. 31 Coexisting unilateral Raynaud's phenomenon with patients of linear scleroderma of the extremities may occur, more often in adults (7%) compared to children (2%). 26,30 In the group of adults patients with unilateral Raynaud phenomenon and linear scleroderma, circulating ANA (antinuclear antibodies) may be found.…”

A retrospective study: Impact of consensus treatment plans on systemic therapy of pediatric morphea

“…Morphea, or localized scleroderma, is an inflammatory and fibrosing disorder of the skin and underlying subcutaneous tissues . Morphea is a distinct disease from systemic scleroderma as it is less commonly associated with internal organ involvement .…”

“…However, up to one‐fourth of children with morphea were found to have extracutaneous manifestations in a multinational study . The pathogenesis of morphea is not well understood, but vascular inflammation and imbalance between collagen production and destruction are thought to be important factors . Morphea is classified into five subtypes: circumscribed, linear, generalized, pansclerotic, and mixed .…”

Linear scleroderma: a series of all clinical variants

“…Morphea, also known as localized scleroderma, is a fibrosing disorder of the skin and subcutaneous tissues, wherein the overabundant collagen deposition destroys adnexal structures and hair follicles [1]. The common clinical variants are circumscribed, generalized, bullous, linear and deep [2].…”