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Inicio Medicina Clínica Práctica Case report of scleromyxedema in a Purtscher-like retinopathy
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Vol. 2. Núm. 2.
Páginas 34 (Marzo - Abril 2019)
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Vol. 2. Núm. 2.
Páginas 34 (Marzo - Abril 2019)
Images in medicine
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Case report of scleromyxedema in a Purtscher-like retinopathy
Reporte de un caso de escleromixedema en una retinopatía tipo Purtscher
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Glenda Espinosa-Barberia,b,
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glenda_eb@hotmail.com

Corresponding author.
, Francisco José Galván Gonzáleza, Evelina Germán Harmannc, Michel Ernesto Valdés Martínd, Miguel Ángel Reyes Rodrígueza, Félix Francisco Hrnándeze
a Doctor Negrín University Hospital, Ophthalmology Department, Las Palmas de Gran Canaria, Las Palmas, Spain
b School of Doctoral and Postgraduate, University of Las Palmas de Gran Canaria, Las Palmas de Gran Canaria, Las Palmas, Spain
c General Hospital of Lanzarote, Ophthalmology Department, Arrecife, Lanzarote, Spain
d Hospiten Clinic Group, Endoluminal/Vascular Department, Santa Cruz de Tenerife, Tenerife, Spain
e Doctor Negrín University Hospital, Rheumatology Department, Las Palmas de Gran Canaria, Las Palmas, Spain
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A 55-year-old woman with a 3-year history of sclerodermiform syndrome undergoing treatment with systemic corticosteroids who presented with a week of low-grade fever, confusional syndrome and visual hallucinations. On physical examination there are white papular lesions that converge forming raised lichenoid zones and between these other hyperpigmented with facial, trunk and limb involvement There was sclerodactyly, microstomy and alopecia in eyebrows (‘Leonine-like’ face) (Fig. 1A and B). The neurological examination showed temporary disorientation, limitation to supraversion and dysarthria. Ophthalmological examination revealed sclerotic and tortuous vessels with whitish lesions, reminiscent of Purtscher's fleckens and hemorrhages affecting the posterior pole (Fig. 1C and D). MRI revealed multiple points of hyperintensity in both hemispheres compatible with lacunar infarcts (Fig. 1E and F). The laboratory tests revealed a globular sedimentation rate of 8.5mm/h and creatinine levels of 5mg/dL, without finding signs of hemolysis, with normal levels of enzyme ADAMTS13, which ruled out microangiopathy of the type thrombocytopenic purpura. The proteinogram presented a monoclonal peak of 9g/L of immunoglobulin G-Kappa. The skin biopsy showed myxoid dermal material with fibroblastic proliferation, while that of bone marrow and the absence of Bence-Jones chains in urine rule out the presence of multiple myeloma. A diagnosis of scleromyxedema with systemic involvement due to microangiopathic thrombotic crisis is reached, and despite the poor prognosis, treatment with plasmapheresis and eculizumab 900mg/week is started, obtaining a two-week improvement in the neurological status and renal disease, which is why the hospital discharge for ambulatory control.

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