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.

Fig. 1

Circumscribed superficial morphea (morphea en plaque). A yellow-white sclerotic plaque with an erythematous or violaceous border, the characteristic ‘lilac ring’

Fig. 2

Generalized morphea. Example of a patient with absence of induration, but extensive hyperpigmentation

Fig. 3

Linear morphea of the upper extremity

Fig. 4

Linear morphea of the lower extremity. Limb length discrepancy and subcutaneous atrophy in burned-out disease (left leg)

Fig. 5

Linear morphea on the paramedian forehead (en coup de sabre) crossing onto the scalp causing alopecia

Fig. 6

Severe case of generalized morphea on the trunk with atrophy of both breasts

Fig. 7

Peau d’orange aspect on upper extremity in a patient with eosinophilic fasciitis

Fig. 8

Flow chart for considerations with regard to diagnosis and monitoring of morphea and eosinophilic fasciitis. EF eosinophilic fasciitis, EMG electromyography, MRI magnetic resonance imaging, SSc systemic sclerosis, US ultrasonography

Fig. 9

Flow chart for the management of morphea and eosinophilic fasciitis. * Topical Corticosteroids (TCS): moderately potent TCS once daily for 3 months. Highly potent TCS once daily for 1 month. ** Topical calcipotriol 0.005% ointment: once or twice daily, with or without occlusion. Possibly in combination with TCS. *** Topical tacrolimus 0.1% ointment: once or twice daily, with or without occlusion. Possibly in combination with TCS. ^† Phototherapy: preferably UVA1; suggested dose: 60 J/cm² to a cumulative dose of 1460 J/cm². If UVA1 is unavailable or impractical, alternative modalities are broadband UVA, PUVA or UVB. Methotrexate (MTX): adult starting dose 15 mg/week, max dose 25 mg/week; pediatric starting dose 15 mg/m², max dose 25 mg. Folic acid supplementation: 0.4–1 mg/day or 5–10 mg/week. Systemic corticosteroids (SCS): adult starting dose 0.5–1 mg/kg/day (max 60 mg) during a max of 3 months followed by tapering; pediatric dose: 1–2 mg/kg/day, max dose 60mg/day, followed by tapering. Intravenous methylprednisolone (IVMP): adult dose 1000 mg/day for 3 days/month for 3–6 months, possibly followed by oral SCS. Pediatric dose 30 mg/kg/day for 3 days/month for 3 months, possibly followed by oral SCS. ^α Mycophenolate Mofetil (MMF, alternative to MTX): adult dose 1000 mg twice daily. Pediatric dose 600–1200 mg/m²/day twice daily. ^A Deep/linear subtypes: treatment with MTX monotherapy. Addition of SCS or IVMP in case of rapidly progressive disease or in the presence of (looming) contractures. ^B Eosinophilic Fasciitis: standard induction treatment with oral SCS or IVMP in combination with MTX . PUVA psoralen plus broadband UVA, UV ultraviolet