Case Report: Pansclerotic Morphea-Clinical Features, Differential Diagnoses and Modern Treatment Concepts.

Details

Serval ID
serval:BIB_F62DE0476303
Type
Article: article from journal or magazin.
Publication sub-type
Case report (case report): feedback on an observation with a short commentary.
Collection
Publications
Institution
Title
Case Report: Pansclerotic Morphea-Clinical Features, Differential Diagnoses and Modern Treatment Concepts.
Journal
Frontiers in immunology
Author(s)
Ventéjou S., Schwieger-Briel A., Nicolai R., Christen-Zaech S., Schnider C., Hofer M., Bogiatzi S., Hohl D., De Benedetti F., Morren M.A.
ISSN
1664-3224 (Electronic)
ISSN-L
1664-3224
Publication state
Published
Issued date
2021
Peer-reviewed
Oui
Volume
12
Pages
656407
Language
english
Notes
Publication types: Case Reports
Publication Status: epublish
Abstract
Pansclerotic morphea (PSM) is a rare skin disease characterized by progressive stiffening of the skin with or without the typical superficial skin changes usually seen in morphea (localized scleroderma). Standard therapy, consisting of a combination of systemic glucocorticoids and methotrexate or mycophenolate mofetil, does rarely stop disease progression, which may lead to severe cutaneous sclerosis and secondary contractures. Little is known about the efficacy of newer biologicals such as abatacept, a fusion protein antibody against CTLA-4, or tocilizumab, a fully humanized IL-6R antibody, in the treatment of this pathology. We present the case of an 8 years old girl with an unusual, progressive stiffening of the skin, which was eventually diagnosed as pansclerotic morphea. A treatment with systemic glucocorticoids and methotrexate combined with tocilizumab led to a good clinical response within 2 months after initiation. In this paper, we discuss differential diagnoses to be considered and this new promising treatment option based on a case review of the literature.
Keywords
IL-6, case report, pansclerotic morphea, scleroderma, stiff skin, tocilizumab
Pubmed
Web of science
Open Access
Yes
Create date
03/04/2021 10:13
Last modification date
13/04/2021 6:36
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