Scleroderma, mixed connective-tissue disease and overlap syndromes: Case

Framing the case

This six-year-old boy has the classic presentation of the linear scleroderma, the commonest subtype of the juvenile localised scleroderma. The band of hard, shiny skin crossing the right knee, the five-month duration, the violaceous active edge, and the loss of the full extension together declare the active linear disease, and the limp is the functional consequence of the tethered joint. The framework that organises the case is the localised scleroderma, the skin-confined family, and the urgent need to halt the active inflammation before the contracture fixes in the growing child. ^[6][7]

Immediate assessment and the classification

The assessment begins with the recognition that this is the localised and not the systemic disease. The absence of the Raynaud phenomenon, the sclerodactyly, the nailfold changes and the systemic symptoms places the lesion in the localised family, and the band morphology places it in the linear subtype. The Zulian classification, drawn from the seven hundred and fifty children, sorts the localised disease into the circumscribed morphea, the linear scleroderma, the generalised morphea, the pansclerotic morphea and the mixed morphea, and the linear subtype is the commonest in children, around two-thirds. The band is photographed and mapped, the joint range is measured, and the limb length is compared for the asymmetry. ^[6][8]