Disabling pansclerotic morphea of childhood with extracutaneous manifestations

Abstract

Disabling pansclerotic morphea (DPM) of childhood is a rare generalized type of localized scleroderma (LS) that is known to follow an aggressive course with pansclerotic lesions leading to severe joint contractures and consequent immobility. Mortality is due to complications of the disease such as bronchopneumonia, sepsis, or gangrene. There is no specific laboratory finding. Treatment protocols are still evolving for this severe recalcitrant disorder. Extracutaneous manifestations are rarely reported in DPM. We present the case of a 7-year-old girl with DPM with severe extracutaneous manifestations in the form of gastrointestinal and vascular disease, whose disease progressed rapidly. In spite of treatment with methotrexate, corticosteroids, and PUVA therapy, she ultimately succumbed to her illness due to sepsis.

Keywords: Disabling pansclerotic morphea of childhood; extracutaneous manifestations; gangrene; juvenile localized scleroderma.

Figure 1

Generalized sclerosis of the skin with joint contractures and crusted erosions on knee

Figure 2

Sclerosis and contractures of hands and feet

Figure 3

Before and after 3 months of methotrexate showing worsening sclerosis with increasing ectropion and lagophthalmos

Figure 4

Cyanosis over toes, which later turned into gangrene