Disabling pansclerotic morphea of childhood--unusual case and management challenges

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.

Fig.1

disseminated hyperpigmented sclero-atrophic plaques on the chest and flanks, moderate atrophy of left breast caused by fibrotic process and discrete limitation of the extension of elbows (maximal 150 degrees)

Fig.2

linear sclero-atrophic lesions on posterior left hemitorax, following Blashko’s lines and on posterior side of left arm

Fig.3

delineation of linear lesions on both inferior limbs

Fig.4

extended hyperpigmented sclero-atrophic plaques on flanks and chest, with indurations and severe atrophy of the left breast and sclerosis involving all the surface of forearms and elbows up to distal third of the arms; fixation of elbows at 90 degrees flexion; fibrous bands extended to the left shoulder

Fig.5

pansclerotic lesions with important atrophy of the underlying muscles and immobilization in semi-flexion of the fingers of both hands and first, with the absence of cutaneous fold; severe limitation of the mobility of the wrists

Fig.6

extended sclero-atrophic lesions with indurations and atrophy on the surface of the legs, with sclerotic bands extending over the knees to the proximal thighs