[Facial linear scleroderma associated with neurological abnormalities relating to microangiopathy].
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.


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.


CONCLUSIONS
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.
Service dermatologie, hôpital Larrey, université Paul Sabatier, CHU, 24, chemin de Pouvourville, TSA 30030, 31059 Toulouse cedex 9, France. Electronic address: laureline.legendre31@gmail.com.
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Legendre
Service de neurologie, hôpital Purpan, université Paul Sabatier, CHU, place du Docteur-Baylac, TSA 40031, 31059 Toulouse cedex 9, France.
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Cuinat
Service de neurologie, hôpital Purpan, université Paul Sabatier, CHU, place du Docteur-Baylac, TSA 40031, 31059 Toulouse cedex 9, France.
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Curot
Service de neuroradiologie, hôpital Purpan, université Paul Sabatier, CHU, place du Docteur-Baylac, TSA 40031, 31059 Toulouse cedex 9, France.
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Tanchoux
Service de neuroradiologie, hôpital Purpan, université Paul Sabatier, CHU, place du Docteur-Baylac, TSA 40031, 31059 Toulouse cedex 9, France.
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Bonneville
Service dermatologie, hôpital Larrey, université Paul Sabatier, CHU, 24, chemin de Pouvourville, TSA 30030, 31059 Toulouse cedex 9, France.
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Mazereeuw-Hautier
D000293:Adolescent; D001763:Blepharoptosis / Q000209:etiology; D059345:Cerebral Small Vessel Diseases / Q000150:complications* / Q000175:diagnosis; D005148:Facial Dermatoses / Q000150:complications*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D010292:Paresthesia / Q000209:etiology; D012594:Scleroderma, Localized / Q000150:complications*
Anomalies neurologiques; Linear scleroderma; Microangiopathie; Microangiopathy; Microbleeding; Microsaignements; Morphea; Morphée; Neurological abnormalities; Ptosis; Sclérodermie linéaire