title: [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.
  
  
  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.
  
  
  CONCLUSIONS
  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.
  
  
  CONCLUSIONS
  Our case highlights the clinical characteristics of this uncommon form of localized scleroderma, the extremely severe prognosis, and the therapeutic challenge involved.
authors: Service de dermatologie, CHU Larrey-Toulouse, 24, chemin de Pouvourville, 31400 Toulouse, France. Electronic address: juliette.marie.hardy@gmail.com.
authors: J
authors: J
authors: Hardy
authors: Service de médecine interne-rhumatologie, hôpital des enfants, 330, avenue de Grande-Bretagne, 31059 Toulouse, France.
authors: C
authors: C
authors: Audouin-Pajot
authors: Service de chirurgie orthopédique, traumatologique et plastique, hôpital des enfants, 330, avenue de Grande-Bretagne, 31059 Toulouse, France.
authors: A
authors: A
authors: Abid
authors: Service d'anesthésie-réanimation, hôpital des enfants, 330, avenue de Grande-Bretagne, 31059 Toulouse, France.
authors: D
authors: D
authors: Chiotasso
authors: Service d'anesthésie-réanimation, hôpital des enfants, 330, avenue de Grande-Bretagne, 31059 Toulouse, France.
authors: B
authors: B
authors: Coustets
authors: Service de douleur et soins palliatifs, hôpital des enfants, 330, avenue de Grande-Bretagne, 31059 Toulouse, France.
authors: A
authors: A
authors: Suc
authors: Service de psychiatrie de l'enfant et de l'adolescent, hôpital La Grave, 7, place Lange, 31300 Toulouse, France.
authors: P
authors: P
authors: Timsit
authors: Service de médecine interne, hôpital Cochin, 27, rue du Faubourg-Saint-Jacques, 75014 Paris, France.
authors: L
authors: L
authors: Mouthon
authors: Service de dermatologie, CHU Larrey-Toulouse, 24, chemin de Pouvourville, 31400 Toulouse, France.
authors: J
authors: J
authors: Mazereeuw-Hautier
mesh_terms: D002100:Cachexia / Q000209:etiology; D002648:Child; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007871:Leg Ulcer / Q000209:etiology; D012594:Scleroderma, Localized / Q000150:complications*; D018805:Sepsis / Q000209:etiology
keywords: Corticosteroids; Corticoïdes; Cyclophosphamide; Extracorporeal photopheresis; Iloprost; Methotrexate; Morphée pansclérotique; Méthotrexate; Pansclerotic morphea; Photochimiothérapie extracorporelle