[Pansclerotic morphea with a rapidly fatal outcome in an 11-year-old girl]

Abstract

Background: Pansclerotic morphea is a poorly described but extremely debilitating variant of localized scleroderma. We report a case with a rapidly fatal outcome in an 11-year-old girl.

Patients and methods: An 11-year-old girl with a 2-year history of morphea presented at our institution in April 2012. The sclerosis had started on her trunk and progressed rapidly to involve her entire skin. Initial treatment with corticosteroids was ineffective and she presented extremely painful ulcerations of the lower limbs. The outcome was rapidly fatal, in early 2014, due to cachexia and sepsis after two amputations and several failed treatments including methotrexate.

Discussion: Pansclerotic morphea is characterized by rapidly progressing sclerosis involving the entire skin, trophic cutaneous ulcers, painful contraction and limited joint mobility. The prognosis is poor since the disease has an incapacitating and potentially fatal outcome. No reliably effective treatment has yet been established.

Conclusion: Our case highlights the clinical characteristics of this uncommon form of localized scleroderma, the extremely severe prognosis, and the therapeutic challenge involved.

Keywords: Corticosteroids; Corticoïdes; Cyclophosphamide; Extracorporeal photopheresis; Iloprost; Methotrexate; Morphée pansclérotique; Méthotrexate; Pansclerotic morphea; Photochimiothérapie extracorporelle.