![]() To our best knowledge, only 2 comparable cohorts, including a large number of patients with LSECDS and PFH, have been published so far (4, 8). There are limited data available on linear LS of the head and face area. Linear LS is the most common subtype in children (28.6–65%), whereas it accounts for only approximately one-tenth of adult-onset cases (7.1–13.8%) (4–7). The demographics and subtype distribution of LS have been well described in several large cohorts, including 4 studies of adult-onset and 6 studies of juvenile LS (for review see Lis-?wi?ty et al. However, there was no history of a tick bite or clinical symptoms of Lyme disease. Six (6.25%) of them had detectable IgM and IgG antibodies. Serological screening for antibodies to Borrelia burgdorferi was performed in 70 (72.9%) of the patients. In the whole cohort, ANA were found most frequently (27.1%), followed by anti-smooth muscle antibodies (11.5%), and extractable nuclear antigens (7.3%). The percentage of positive autoimmune parameters was similar in patients with LSECDS (64.3%) and PFH (62.5%). All 10 (100.0%) patients with LSECDS/PFH overlap had at least 1 positive parameter (total: 18 parameters). Overall, 73 (76.0%) of the patients revealed blood parameters of autoimmunity ( Table SIV). Psychiatric symptoms were not observed in any of the patients.Īmong the 96 patients, 14 (14.6%) had other rheumatic diseases (1 case of rheumatoid arthritis and 1 case of spondyloarthropathy) or other autoimmune disorders (1 case each of lichen sclerosus, linear IgA-dermatosis, multiple sclerosis, primary biliary cholangitis, and systemic lupus erythematosus 2 cases of alopecia areata 5 cases of autoimmune thyroiditis). Most frequent MRI and CT abnormalities were gliosis (7.3%) and white matter lesions (6.3%). Headache (5.2%) was the most frequently presented symptom, followed by epilepsy (4.2%) and migraine (3.1%). These abnormalities were most common in LSECDS/PFH overlap (80%), followed by PFH (37.5%) and LSECDS (18.6%). Overall, 27 (28.1%) of the cohort presented neurological symptoms and/or radiological CNS abnormalities. MRI and computed tomography (CT) evaluations were available in 52 (54.2%) of the 96 patients ( Table SIII). The most frequently affected site of extrafacial LS lesions was the trunk (8.3%), followed by the neck (4.2%) ( Table SII). ![]() Extrafacial disease was more frequent in patients with PFH (37.5%) and LSECDS/PFH overlap (40%) compared with patients with LSECDS (20%). Of the whole cohort, 24 (25%) patients presented extrafacial LS lesions at initial diagnosis. Mean ± standard deviation age at first diagnosis was 21.9 ± 16.4 years (range 1–68) ( Table SI). The female to male ratio was 2.5:1, with 68 female patients (25 girls and 43 adult women) and 28 male patients (11 boys and 17 adult men). Of these, 70 patients had LSECDS, 16 had PFH, and 10 overlapping LSECDS and PFH. A detailed description of the methodology is shown in Appendix S1.Ī total of 96 patients with head and face LS was included in this retrospective analysis. The purpose of this study was to evaluate clinical, radiological and laboratory characteristics in a large cohort of patients with LSECDS, PFH, or overlap of both diseases.Ī retrospective review was performed using the digital databases of LS patients with linear subtypes, such as LSECDS, PFH, or a combination of both types. As neurological involvement and abnormalities in the central nervous system (CNS) are frequent in LSECDS and PFH, current guidelines recommend magnetic resonance imaging (MRI), irrespective of the presence of clinical symptoms (1, 2). Although large studies already exist on the demographic characteristics and subtype distribution in both adult and juvenile LS, there are fewer data available on the clinical findings of LSECDS and PFH, especially in patients with adult-onset disease (3). LS “en coup de sabre” (LSECDS) and progressive facial hemiatrophy (PFH) are rare subtypes located on the head and face area, belonging to the group of linear LS. This guideline distinguishes 5 main subtypes (limited, generalized, linear, deep, and mixed) and considers eosinophilic fasciitis as a separate type within the spectrum of LS (2). A recently published European Dermatology Forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin includes a classification based on the current German guidelines for LS (1, 2). ![]() Localized scleroderma (LS) is a relatively rare sclerotic auto-immune disease that primarily affects the skin, but might also involve adjacent tissues, such as the fat tissue, fascia, muscle, and bone.
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