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Advances in dermatology and venereology Acta Dermato-Venereologica
Localized Scleroderma of the Head and Face Area : A Retrospective Cross-sectional Study of 96 Patients from 5 German Tertiary Referral Centres
Alexander KREUTER 1 *, Georg MITRAKOS 1 , Silke C . HOFMANN 2 , Percy LEHMANN 2 , Michael STICHERLING 3 , Thomas KRIEG 4 , Nina LAHNER 5 , Christian TIGGES 1 , Nicolas HUNZELMANN 4 and Pia MOINZADEH 4
1
Department of Dermatology , Venereology , and Allergology , HELIOS St Elisabeth Hospital Oberhausen , University Witten-Herdecke ,
2
Department of Dermatology , Allergology , and Dermatosurgery , HELIOS University Hospital Wuppertal , University of Witten / Herdecke , Departments of Dermatology , 3 Universitätsklinikum Erlangen , Friedrich-Alexander-University Erlangen-Nürnberg , Erlangen , 4 University of Cologne , Köln , and 5 Ruhr-University , Bochum , Germany . * E-mail : alexander . kreuter @ helios-gesundheit . de Accepted Mar 1 , 2018 , Epub ahead of print Mar 2 , 2018
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 . 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 ). 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 ).
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 . 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 ). 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 ). 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 .
MATERIALS , METHODS AND RESULTS
A retrospective review was performed using the digital databases of LS patients with linear subtypes , such as LSECDS , PFH , or a combination of both types . A detailed description of the methodology is shown in Appendix S1 1 .
A total of 96 patients with head and face LS was included in this retrospective analysis . Of these , 70 patients had LSECDS , 16 had PFH , and 10 overlapping LSECDS and PFH . 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 ). Mean ± standard deviation age at first diagnosis was 21.9 ± 16.4 years ( range 1 – 68 ) ( Table SI 1 ).
Of the whole cohort , 24 ( 25 %) patients presented extrafacial LS lesions at initial diagnosis . Extrafacial disease was more frequent in patients with PFH ( 37.5 %) and LSECDS / PFH overlap ( 40 %) compared with patients with LSECDS ( 20 %). The most frequently affected site of extrafacial LS lesions was the trunk ( 8.3 %), followed by the neck ( 4.2 %) ( Table SII 1 ).
1 https :// www . medicaljournals . se / acta / content / abstract / 10.2340 / 00015555-2920
MRI and computed tomography ( CT ) evaluations were available in 52 ( 54.2 %) of the 96 patients ( Table SIII 1 ). Overall , 27 ( 28.1 %) of the cohort presented neurological symptoms and / or radiological CNS abnormalities . These abnormalities were most common in LSECDS / PFH overlap ( 80 %), followed by PFH ( 37.5 %) and LSECDS ( 18.6 %). Headache ( 5.2 %) was the most frequently presented symptom , followed by epilepsy ( 4.2 %) and migraine ( 3.1 %). Most frequent MRI and CT abnormalities were gliosis ( 7.3 %) and white matter lesions ( 6.3 %). Psychiatric symptoms were not observed in any of the patients .
Among 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 ). Overall , 73 ( 76.0 %) of the patients revealed blood parameters of autoimmunity ( Table SIV 1 ). All 10 ( 100.0 %) patients with LSECDS / PFH overlap had at least 1 positive parameter ( total : 18 parameters ). The percentage of positive autoimmune parameters was similar in patients with LSECDS ( 64.3 %) and PFH ( 62.5 %). 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 %). Serological screening for antibodies to Borrelia burgdorferi was performed in 70 ( 72.9 %) of the patients . Six ( 6.25 %) of them had detectable IgM and IgG antibodies . However , there was no history of a tick bite or clinical symptoms of Lyme disease .
DISCUSSION
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-Święty et al . ( 3 )). 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 ). There are limited data available on linear LS of the head and face area . 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 ). Zulian et al . ( 4 ) evaluated 113 patients ( 99 with LSECDS , 8 with PFH and 6 with LSECDS / PFH overlap ) by retrospectively collecting the data of 70 paediatric dermatology and rheumatology centres from all over the world . Doolittle et al . ( 8 ) performed a retrospective single centre analysis over a 16-year interval and reported the CNS imaging findings of 88 patients with head and face LS ( 43 with LSECDS , 30 with PFH and 15 with LSECDS / PFH overlap ). In this study , a substantial proportion ( 25.0 %) of patients presented LS lesions outside of the head and face
This is an open access article under the CC BY-NC license . www . medicaljournals . se / acta Journal Compilation © 2018 Acta Dermato-Venereologica . doi : 10.2340 / 00015555-2920 Acta Derm Venereol ; 2018 ; 98 : 603 – 605