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Disabling pansclerotic morphea of childhood – unusual case and management challenges

Forsea Ana-Maria,Cretu Aura-Nicoleta,Ionescu Ruxandra,Giurcaneanu Calin

Published 2008 in Journal of Medicine and Life

ABSTRACT

Morphea, also known as localized scleroderma is a chronic disease of unknown etiology, characterized by fibrous deposition and obliteration of vessels in the skin. This disease has a wide clinical spectrum, ranging from mild hyperpigmented plaques to severe, invalidating generalized and pansclerotic forms. Disabling pansclerotic morphea of childhood is a rare and debilitating variant of localized scleroderma, characterized by a rapid progression of deep cutaneous fibrosis that involves the dermis and the subcutaneous adipose tissue but also fascia, muscles, and bone. Contractures and musculoskeletal atrophy develop and the disease has an invalidating and even fatal course. We present an unusual case of severe morphea in a 19-year-old girl, with a polymorphous clinical picture consisting of plaques, linear and pansclerotic, circumferential lesions, with symmetric, invalidating involvement of all limbs and explosive evolution with centripetal progression. This case emphasizes the unpredictable character of morphea evolution, the possible severe prognosis and the therapeutic challenges raised by the generalized, disabling forms of this disease.

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