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

  • Asya I. WallachEmail author
  • Cynthia M. Magro
  • Andrew G. FranksJr
  • Lee Shapiro
  • Ilya Kister
Neurology of Systemic Diseases (J Biller, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Neurology of Systemic Disease

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.

Summary

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

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

Notes

Compliance with Ethical Standards

Conflict of Interest

Asya Wallach reports educational grants from the National MS Society and Biogen. Lee Shapiro reports he served on advisory board for Genentech. Ilya Kister reports he served on advisory boards for Biogen and Genentech and received research support for investigator-initiated grants from Sanofi Genzyme, Biogen, EMD Serono, National MS Society, and Guthy Jackson Charitable Foundation. Cynthia Magro and Andrew Franks each declare no potential conflicts of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  • Asya I. Wallach
    • 1
    Email author
  • Cynthia M. Magro
    • 2
  • Andrew G. FranksJr
    • 3
    • 4
  • Lee Shapiro
    • 5
  • Ilya Kister
    • 1
  1. 1.NYU Multiple Sclerosis Comprehensive Care Center, Department of NeurologyNew York University School of MedicineNew YorkUSA
  2. 2.Department of Pathology and Laboratory MedicineWeill Cornell MedicineNew YorkUSA
  3. 3.Skin Lupus & Autoimmune Connective Tissue Section, The Ronald O. Perelman Department of DermatologyNew York University School of MedicineNew YorkUSA
  4. 4.Division of Rheumatology, Department of MedicineNew York University School of MedicineNew YorkUSA
  5. 5.Community Care RheumatologySaratoga SpringsUSA

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