[Facial linear scleroderma associated with neurological abnormalities relating to microangiopathy]. / Sclérodermie linéaire de la face associée à des anomalies neurologiques par microangiopathie.

BACKGROUND: Linear scleroderma is a fibrotic disease affecting the skin and sometimes the deeper tissues. We describe a case of scleroderma associated with neurological anomalies not previously reported in the literature. PATIENTS AND METHODS: A 16-year-old male patient presented in 2009 for hemifacial linear scleroderma. Treatment with methotrexate for 14 months resulted in stabilization of the disease. In 2013, we noted worsening of the patient's skin lesions as well as homolateral ptosis. Head MRI revealed unilateral hemispherical signal abnormalities with T2 hypersignal in the basal gangliaand punctate foci of T2* hyposignal corresponding to microbleeds. In 2014 and 2015, the patient presented three brief episodes of right hemicorpus paresthesia (with temporary aphasia followed by headache during the first episode). The head MRI showed worsening of the anomalies, suggesting progressing cerebral microangiopathy. DISCUSSION: Clinicians may not always be familiar with the neurological abnormalities associated with localized facial scleroderma even if such abnormalities are not uncommon (their exact prevalence is unknown). Clinical signs vary but, in most cases, the radiological features are calcifications and hyperintense foci of white matter lesions in T2. As far as we are aware, there have been no reports to date of microbleeding as observed in our patient. The worsening with time of these neurological anomalies of unknown origin does not appear to be correlated with the dermatological lesions. It is important for dermatologists be aware of these complications of facial linear scleroderma.

[Pansclerotic morphea with a rapidly fatal outcome in an 11-year-old girl]. / Morphée pansclérotique d'évolution rapidement létale chez une jeune fille de 11ans.

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.

[Eosinophilic fasciitis, morphea and vitiligo in a single patient]. / Association fasciite avec éosinophilie, morphées et vitiligo.

BACKGROUND: Fasciitis with eosinophilia (FE), or Shulman syndrome, is a rare disease of unknown origin for which the nosological profile has not been clearly defined. It is clinically characterised by oedema and induration of the limbs with hypereosinophilia. It may be associated with morphea, in which case it carries a poor prognosis, or other diseases, particularly autoimmune conditions. Herein, we report a case of fasciitis associated with eosinophilia, morphea and vitiligo. PATIENT AND METHODS: A 45-year-old male patient followed up for vitiligo for 20 years had been presenting swelling and induration of the skin on all 4 limbs for the previous 7 months associated with morphea on the trunk. Treatment consisting of systemic corticosteroids and methotrexate was initiated and displayed a certain degree of efficacy. DISCUSSION: The association of morphea/fasciitis with eosinophilia is a classical finding; the presence of vitiligo raises the question of possible association between these different disorders.

[Radio-induced breast morphoea: an unrecognized complication]. / Morphée du sein radio-induite : une complication méconnue.

Morphoea is a localized scleroderma. Since the 1990s, it is described as a complication after irradiation for breast cancer. This complication is unrecognized and underdiagnosed. Irradiation seems to be a factor inducing an autoimmune process. Clinicians should be aware in case of an erythema in a treated area, appearing on average one year after the end of the irradiation. Histology alone can prove the diagnosis and rule out differential diagnoses: principally mastitis (carcinomatous, infectious), chronic radiodermatitis or radiation recall. Treatment of this rare complication is not consensual; it is most often topical steroids. The evolution is marked by a gradual decrease from the initial inflammation. Sclerosis persists, more or less substantially.