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2017 ; 18
(4
): 491-512
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Morphea and Eosinophilic Fasciitis: An Update
#MMPMID28303481
Mertens JS
; Seyger MMB
; Thurlings RM
; Radstake TRDJ
; de Jong EMGJ
Am J Clin Dermatol
2017[Aug]; 18
(4
): 491-512
PMID28303481
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Morphea, also known as localized scleroderma, encompasses a group of idiopathic
sclerotic skin diseases. The spectrum ranges from relatively mild phenotypes,
which generally cause few problems besides local discomfort and visible
disfigurement, to subtypes with severe complications such as joint contractures
and limb length discrepancies. Eosinophilic fasciitis (EF, Shulman syndrome) is
often regarded as belonging to the severe end of the morphea spectrum. The exact
driving mechanisms behind morphea and EF pathogenesis remain to be elucidated.
However, extensive extracellular matrix formation and autoimmune dysfunction are
thought to be key pathogenic processes. Likewise, these processes are considered
essential in systemic sclerosis (SSc) pathogenesis. In addition, similarities in
clinical presentation between morphea and SSc have led to many theories about
their relatedness. Importantly, morphea may be differentiated from SSc based on
absence of sclerodactyly, Raynaud's phenomenon, and nailfold capillary changes.
The diagnosis of morphea is often based on characteristic clinical findings.
Histopathological evaluation of skin biopsies and laboratory tests are not
necessary in the majority of morphea cases. However, full-thickness skin
biopsies, containing fascia and muscle tissue, are required for the diagnosis of
EF. Monitoring of disease activity and damage, especially of subcutaneous
involvement, is one of the most challenging aspects of morphea care. Therefore,
data harmonization is crucial for optimizing standard care and for comparability
of study results. Recently, the localized scleroderma cutaneous assessment tool
(LoSCAT) has been developed and validated for morphea. The LoSCAT is currently
the most widely reported outcome measure for morphea. Care providers should take
disease subtype, degree of activity, depth of involvement, and quality-of-life
impairments into account when initiating treatment. In most patients with
circumscribed superficial subtypes, treatment with topical therapies suffices. In
more widespread disease, UVA1 phototherapy or systemic treatment with
methotrexate (MTX), with or without a systemic corticosteroid combination, should
be initiated. Disappointingly, few alternatives for MTX have been described and
additional research is still needed to optimize treatment for these debilitating
conditions. In this review, we present a state-of-the-art flow chart that guides
care providers in the treatment of morphea and EF.
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