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Curr Neurol Neurosci Rep. 2019 Feb 12;19(3):11. doi: 10.1007/s11910-019-0929-8.

Protean Neurologic Manifestations of Two Rare Dermatologic Disorders: Sweet Disease and Localized Craniofacial Scleroderma.

Author information

1
NYU Multiple Sclerosis Comprehensive Care Center, Department of Neurology, New York University School of Medicine, New York, NY, USA. asyawallach@gmail.com.
2
Department of Pathology and Laboratory Medicine, Weill Cornell Medicine, New York, NY, USA.
3
Skin Lupus & Autoimmune Connective Tissue Section, The Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, NY, USA.
4
Division of Rheumatology, Department of Medicine, New York University School of Medicine, New York, NY, USA.
5
Community Care Rheumatology, Saratoga Springs, NY, USA.
6
NYU Multiple Sclerosis Comprehensive Care Center, Department of Neurology, New York University School of Medicine, New York, NY, USA.

Abstract

PURPOSE OF REVIEW:

To describe diverse neurologic and neuroradiologic presentations of two rare, immunologically mediated skin conditions: Sweet disease and localized scleroderma (morphea).

RECENT FINDINGS:

Core syndromes of neuro-Sweet disease (NSD) are steroid responsiveness, recurrent meningitis, and encephalitis. Focal neurologic, neuro-vascular, and neuro-ophthalmologic syndromes have been reported recently in NSD. A variety of steroid-sparing treatments and biologics have been used for relapsing NSD. Localized craniofacial scleroderma is associated with seizures, headaches, and, less commonly, focal deficits and cognitive decline. Immunosuppressive therapy may be required in patients with disease progression; some refractory cases have responded to IL-6 inhibition. Our review provides an up-to-date reference for neurologists faced with a patient with a history or skin findings consistent with Sweet disease or localized scleroderma. We hope that it will stimulate collaborative studies aimed at unraveling the pathogenesis of these disorders, better characterization of their neurologic manifestations, and discovery of optimal therapeutic solutions.

KEYWORDS:

Anti-Il 6 therapy; Localized scleroderma; Neuro-Sweet disease; Neurologic complications; Progressive hemifacial atrophy; Sweet syndrome

PMID:
30747288
DOI:
10.1007/s11910-019-0929-8

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