Morphea and Eosinophilic Fasciitis: An Update

Abstract

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.

Juvenile case of multiple morphea profunda resulting in joint contracture that was successfully treated with cyclosporin A: A case report and review of the published works

“…Methotrexate is considered a first‐line treatment and additional systemic corticosteroids are also recommended in severe cases with rapid progression and/or contractures . Oral steroids, employed in two‐thirds of juvenile cases with deeply spread morphea, resolved the joint contractures in our case.…”

Eosinophile Hautkrankheiten

“…Besteht die Erkrankung schon länger oder wurde eine Therapie begonnen, können die eosinophilen Granulozyten auch fehlen. Häufig kann eine begleitende Myositis nachgewiesen werden . Darüber hinaus besteht eine Verdickung der Kollagenfasern in der Dermis.…”

A long history of widespread asymptomatic giant plaques

“…In addition, the differential diagnosis also included superfi cial morphea. However, the fi brotic evolution of epidermis and dermis typical of morphea was not detected on histology; hence, this diagnosis too was dismissed [ 5 ] . Scaly papulosquamous or annular/polycyclic plaques of subacute cutaneous lupus erythematosus (SCLE) should also be considered in the differential diagnosis.…”