Neurological and psychological aspects of scleroderma of the face and neck in children

In the structure of morbidity, among rheumatic diseases in children, juvenile scleroderma takes the third place in frequency after juvenile idiopathic arthritis and systemic lupus erythematosus. Timely diagnosis of juvenile scleroderma presents certain difficulties, since the traditional criteria for making a diagnosis of scleroderma are applicable to adult patients. Unlike adult patients, in the initial period of the disease, lesions of the internal organs are less likely to occur, and joint damage occurs with equal frequency. According to recent studies, the most common neurological manifestations in juvenile scleroderma with localization of lesions on the head and neck are epileptic seizures, headaches, local neurological deficits, psychoemotional and cognitive impairments. In the course of this study, 51 children with scleroderma with localization of lesions on the head and neck at the age of 3 to 17 years were examined. In most patients (80%), the first symptom of the disease was a characteristic skin lesion in the form of a purple spot, followed by linear tissue induction, less often the disease began with the appearance of an alopecia on the scalp or in the eyebrow area. Scleroderma with lesions on the head and neck is the most severe form of all localized types of scleroderma and can be equated in its severity with its generalized forms. The palette of polysystemic disorders in this form requires a multidisciplinary approach in examination and therapy. The list of mandatory examination methods for scleroderma of the face and neck should include brain MRI, EEG and psychological examination. 

1. Zulian F. Scleroderma in children // Best Practice & Research Clinical Rheumatology. 2017; 31, (4): 439-610.

2. Herrick A. L., Ennis H., Bhushan M., Silman A. J., Baildam E. M. Incidence of childhood linear scleroderma and systemic sclerosis // Arthritis Care Res. 2010; 62: 213e8.

3. Zulian F., Martini G. Childhood systemic sclerosis // Current Opinion in Rheumatology. 2007; 19 (6): 592-597.

4. Martini G., Foeldvari I., Russo R., Cuttica R., Eberhard A., Ravelli A., et al. Systemic sclerosis in childhood: clinical and immunological features of 153 patients in an international database // Arthritis Rheum. 2006; 54: 3971-3978.

5. Preliminary criteria for the classification of systemic sclerosis (scleroderma). Committee, The Subcommittee for Scleroderma Criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria // Arthritis Rheum. 1980; 23: 581-590.

6. Amaral T. M., Peres F. A., Lapa A. T., Marques-Neto J. F., Appenzeller S. Neurological involvement inscleroderma: a systematic review // Semin Arthritis. 2013; 43: 335-347.

7. English S. W., Ho M. L., Tollefson M. M., Wong-Kisiel L. C. Focal Epilepsy in a Teenager With Facial Atrophy and Hair Loss // Semin Pediatr Neurol. 2018; 26: 68-73. DOI: 10.1016/j.spen.2017.03.009. Epub 2017 Apr 2.

8. Rocha R., Kaliakatsos M. Epilepsy in paediatric patients with Parry-Romberg syndrome: A review of the literature // Seizure - European Journal of Epilepsy. 2020, January 27. DOI: https://doi.org/10.1016/j.seizure.2020.01.017.

9. Беляева Л. М., Чижевская И. Д. Системный склероз и ограниченная склеродермия у детей и подростков // Педиатрия. Журнал им. Г. Н. Сперанского. 2009, № 5. @@Belyayeva L. M., Chizhevskaya I. D. Sistemnyy skleroz i ogranichennaya sklerodermiya u detey i podrostkov (Systemic sclerosis and limited scleroderma in children and adolescents.) Pediatriya. Zhurnal im. G. N. Speranskogo. 2009, № 5.

10. Baubet T., Ranque B., Taieb O., et al. Mood and anxiety disorders in systemic sclerosis patients // Presse Med. 2011; 40: e111-119.

11. Blaszczyk M., Krolicki L., Krasu M., Glinska O., Jablonska S. Progressive facial hemiatrophy: central nervous system involvement and relationship with scleroderma en coup de sabre // J Rheumatol. 2003; 30 (9): 1997-2004. [Epub2003/09/11].

12. Jewett L. R., Razykov I., Hudson M., et al, Canadian Scleroderma Research Group. Prevalence of current, 12-month and lifetime major depressive disorder among patients with systemic sclerosis // Rheumatology. 2014; 53: 1719.

13. Kwakkenbos L., Delisle V. C., Fox R. S., Gholizadeh S., Jewett L. R., Levis B., Milette K., Mills S. D., Malcarne V. L., Thombs B. D. Psychosocial Aspects of Scleroderma // Rheum Dis Clin North Am. 2015; 41 (3): 519-528. DOI: 10.1016/j.rdc.2015.04.010. Epub 2015 May 27.

14. Bragazzi N. L., Watadd A., Gizuntermang A., McGonagle D., Mahagnad H., Comaneshterh D., Amitald H., Cohen A. D., Amital D. The burden of depression in systemic sclerosis patients: a nationwide population-based study // Journal of Affective Disorders. 2019; 243: 427-431.

15. Paprocka J., Jamroz E., Adamek D., Marszal E., Mandera M. Difficulties in differentiation entiation of Parry-Romberg syndrome, unilateral facial sclerodermia, and Rasmussen syndrome // Childs Nerv Syst. 2006; 22 (4): 409-415 [Epub2005/10/26].

16. Verhelst H. E., Beele H., Joos R., Vanneuville B., Van Coster R. N. Hippocampal atrophy and developmental regression as first sign of linear scleroderma «en coup desabre» // Eur J Paediatr Neurol. 2008; 12 (6): 508-511 [Epub2008/01/22].

17. Anderson L. E., Treat J. R., Licht D. J., Kreiger P. A., Knight A. M. Remission of seizures with immunosuppressive therapy in Parry?Romberg syndrome anden coup de sabre linear scleroderma: Case report and briefreview of the literature // Pediatr Dermatol. 2018, and 00: 1-3.

18. Fain E. T., Mannion M., Pope E., Young D. W., Laxer R. M., Cron R. Q. Brain cavernomas associated with en coup de sabre linear scleroderma: two case reports // Pediatr Rheumatol Online J. 2011; 9: 18.

19. Korkmaz C., Adapinar B., Uysal S. Beneficial effect of immunosuppressive drugs on Parry-Romberg syndrome: a case report and review of the literature // South Med J. 2005; 98.

20. Осминина М. К., Геппе Н. А. Вопросы классификации, клиническая картина и базисная терапия ювенильной склеродермии // Научно-практическая ревматология. 2015; 53(2): 214-219. https://doi.org/10.14412/1995-4484-2015-214-219. @@Osminina M. K., Geppe N. A. Voprosy klassifikatsii, klinicheskaya kartina i bazisnaya terapiya yuvenil’noy sklerodermii (Classification questions, clinical picture and basic therapy of juvenile scleroderma.) Nauchno-prakticheskaya revmatologiya. 2015; 53(2): pp 214-219. https://doi.org/10.14412/1995-4484-2015-214-219.

21. Kowal-Bielecka O., Fransen J., Avouac J., Becker M., Kulak A., Allanore Y., et al. Update of EULAR recommendations for the treatment of systemic sclerosis. Ann Rheum Dis 2017;76:1327-39.

22. Lythgoe H., Baildam E., Beresford M. W., Cleary G., McCann L. J., Pain C. E. Tocilizumab as a potential therapeutic option for children with severe, refractory juvenile localized scleroderma // Rheumatology (Oxford). 2018; 57 (2): 398-401. DOI: 10.1093/rheumatology/kex382.

23. Magro C. M., Halteh P., Olson L. C., Kister I., Shapiro L. Linear scleroderma «en coup de sabre» with extensive brain involvement-Clinicopathologic correlations and response to anti-Interleukin-6 therapy // Orphanet J Rare Dis. 2019; 14 (1): 110. DOI: 10.1186/s13023-019-1015-7.

24. Tashkin D. P., Roth M. D., Clements P. J., Furst D. E., Khanna D., Kleerup E. C., et al. Mycophenolate mofetil versus oral cyclophosphamide in scleroderma-related interstitial lung disease (SLS II): a randomised controlled, double-blind, parallel group trial // Lancet Respir Med. 2016; 4: 708-719.

25. Beck A. T. et al. An Inventory for Measuring Depression //Archives of general psychiatry. - 1961. - Т. 4. - №. 6. - С. 561-571.

26. Beck AT, Epstein N, Brown G, Steer RA (1988). «An inventory for measuring clinical anxiety: Psychometric properties». Journal of Consulting and Clinical Psychology. 56 (6): 893-897. doi:10.1037/0022-006x.56.6.893.

27. Шкала явной тревожности для детей 8-12 лет (CMAS) / пер. А.М. Прихожан; отв. за выпуск В.Э. Пахальян. - Москва: Лаборатория научных основ детской практической психологии Психологического института РАО, 1994. - (Диагностический банк школьного психолога; выпуск 9) @@Shkala yavnoy trevozhnosti dlya detey 8-12 let (CMAS) (The apparent anxiety scale for children 8-12 years old (CMAS)) per. A.M. Prikhozhan; otv. za vypusk V.E. Pakhal’yan. - Moskva: Laboratoriya nauchnykh osnov detskoy prakticheskoy psikhologii Psikhologicheskogo instituta RAO, 1994. - (Diagnosticheskiy bank shkol’nogo psikhologa; vypusk 9)

28. Тест тревожности (Р.Тэммл, М.Дорки, В.Амен) / Дерманова И.Б. Диагностика эмоционально-нравственного развития - СПб., 2002. С.19-28. @@Test trevozhnosti (R.Temml, M.Dorki, V.Amen) (Anxiety test) Dermanova I.B. Diagnostika emotsional’no-nravstvennogo razvitiya - SPb., 2002. pp.19-28.

29. Векслер Д. Шкала интеллекта Векслера для детей. WISC Wechsler Intelligence Scale for Children. Руководство. Челябинск 2003. @@Veksler D. Shkala intellekta Vekslera dlya detey. WISC Wechsler Intelligence Scale for Children. Rukovodstvo. Chelyabinsk 2003.

30. Чижевская И. Д., Беляева Л. М. Психологические особенности детей с различными формами склеродермии // Научно-практическая ревматология. 2006, № 2. @@Chizhevskaya I. D., Belyayeva L. M. Psikhologicheskiye osobennosti detey s razlichnymi formami sklerodermii (Psychological features of children with various forms of scleroderma.) Nauchno-prakticheskaya revmatologiya. 2006, № 2.

31. Stasiulis E., Gladstone B., Boydell K., O’Brien C., Pope E., Laxer RM. Children with facial morphoea managing everyday life: a qualitative study // Br J Dermatol. 2018; 179 (2): 353-361. DOI: 10.1111/bjd.16449. Epub 2018 Jun 7.

32. Sierakowska M., Doroszkiewicz H., Sierakowska J., Olesi?ska M., Grabowska-Jodkowska A., Brzosko M., Leszczyski P., Pawlak-Bu? K., Batko B., Wiland P., Majdan M., Bykowska-Sochacka M., Romanowski W., Zon-Giebel A., Jeka S., Ndosi M. Factors associated with quality of life in systemic sclerosis: a cross-sectional study // Qual Life Res. 2019; 28 (12): 3347-3354. DOI: 10.1007/s11136-019-02284-9. Epub 2019 Sep 3.