r-n-jРоссийский неврологический журналRussian neurological journal2658-79472686-7192МИА10.30629/2658-7947-2023-28-3-50-54r-n-j-432Research ArticleИССЛЕДОВАНИЯ И КЛИНИЧЕСКИЕ НАБЛЮДЕНИЯCLINICAL RESEARCHES AND CASE REPORTSНеврологические проявления болезни ДегосаNeurological manifestations of Degos diseasehttps://orcid.org/0000-0002-7217-6940АбзаловаД. И.AbzalovaD. I.

Абзалова Диляра Ирековна

Москва

Abzalova Dilyara Irekovna

Moscow

Dila1307@yandex.ruhttps://orcid.org/0000-0001-5026-0060СинкинМ. В.SinkinM. V.

Москва

Moscow

mvsinkin@gmail.comhttps://orcid.org/0000-0002-7864-7483КисельО. В.KisyelO. V.

Москва

Moscow

zh-woman@yandex.ruhttps://orcid.org/0000-0003-3515-8329ГриньА. А.GrinA. A.

Москва

Moscow

aagreen@yandex.ruНаучно-исследовательский институт скорой помощи им. Н. В. СклифосовскогоРоссияN. V. Sklifosovsky Research Institute of Emergency MedicineRussian Federation2023190720232835054Copyright © Абзалова Д.И., Синкин М.В., Кисель О.В., Гринь А.А., 20232023Абзалова Д.И., Синкин М.В., Кисель О.В., Гринь А.А.Abzalova D.I., Sinkin M.V., Kisyel O.V., Grin A.A.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.r-n-j.com/jour/article/view/432

Болезнь Дегоса (БД) – редкое заболевание, проявляющееся мультифокальной васкулопатией, следствием которой является поражение многих систем, в том числе нервной. Это заболевание затруднительно в диагностике, на сегодняшний день не имеет стандартизированного подхода к лечению.

Представлено клиническое наблюдение пациентки 66 лет, дебютом заболевания у которой стало поражение кожных покровов. Причиной госпитализации больной явилось поражение центральной нервной системы. В ходе дообследования пациентки был установлен диагноз – ишемический инсульт в бассейне левых средней мозговой, задней мозговой артерий, правой средней мозговой артерии другой установленной этиологии по TOAST, асептический лептоменингит, пахименингит, возникшие на фоне основного заболевания. Течение БД осложнилось поражением желудочно-кишечного тракта, сердечно-сосудистой системы, а также присоединением коронавирусной инфекции, что привело к летальному исходу.

Degos disease is a rare disease that manifests itself as multifocal vasculopathy, the consequence of which is the defeat of many systems, including the nervous system. This disease is difficult to diagnose, today it does not have a standardized approach to treatment, and it is deadly.

In the presented clinical case, the course of BD is described in a 66-year-old patient, whose debut of the disease was a lesion of the skin. The cause of hospitalization of the patient was the defeat of the central nervous system. During the additional examination, the patient was diagnosed with ischemic stroke in the basin of the left middle cerebral artery, posterior cerebral artery, right middle cerebral artery of another established etiology according to TOAST, aseptic leptomeningitis, pachymeningitis. The course of the disease was complicated by damage to the gastrointestinal tract, the cardiovascular system, as well as the addition of a coronavirus infection, which led to death.

: болезнь Дегосазлокачественный атрофический папулезлетальный кожно-кишечный синдромDegos diseasemalignant atrophic papulosislethal skin-intestinal syndromeReferencesTheodoridis A, Makrantonaki E, Zouboulis CC. Malignant atrophic papulosis (Köhlmeier–Degos disease) — a review. Orphanet J Rare Dis. 2013;8:10. https://doi.org/10.1186/1750-1172-8-10Theodoridis A, Makrantonaki E, Zouboulis CC. Malignant atrophic papulosis (Köhlmeier–Degos disease) — a review. Orphanet J Rare Dis. 2013;8:10. https://doi.org/10.1186/1750-1172-8-10Theodoridis A, Konstantinidou A, Makrantonaki E, Zouboulis CC. Malignant and benign forms of atrophic papulosis (Köhlmeier–Degos disease): systemic involvement determines the prognosis. Br J Dermatol. 2014;170(1):110–115. https://doi.org/10.1111/bjd.12642Theodoridis A, Konstantinidou A, Makrantonaki E, Zouboulis CC. Malignant and benign forms of atrophic papulosis (Köhlmeier–Degos disease): systemic involvement determines the prognosis. Br J Dermatol. 2014;170(1):110–115. https://doi.org/10.1111/bjd.12642Ball E, Newburger A, Ackerman AB. Degos’ disease: a distinctive pattern of disease, chiefly of lupus erythematosus, and not a specific disease perse. Am J Dermatopathol. 2003;25(4):308–320. https://doi.org/10.1097/00000372-200308000-00005Ball E, Newburger A, Ackerman AB. Degos’ disease: a distinctive pattern of disease, chiefly of lupus erythematosus, and not a specific disease perse. Am J Dermatopathol. 2003;25(4):308–320. https://doi.org/10.1097/00000372-200308000-00005Lu JD, Sachdeva M, Silverberg OM, Shapiro L, Croitoru D, Levy R. Clinical and laboratory prognosticators of atrophic papulosis (Degos disease): a systematic review. Orphanet J Rare Dis. 2021;16(1):203. https://doi.org/10.1186/s13023-021-01819-zLu JD, Sachdeva M, Silverberg OM, Shapiro L, Croitoru D, Levy R. Clinical and laboratory prognosticators of atrophic papulosis (Degos disease): a systematic review. Orphanet J Rare Dis. 2021;16(1):203. https://doi.org/10.1186/s13023-021-01819-zБеренбейн БА, Студницин АА. (ред.) Дифференциальная диагностика кожных болезней. Руководство для врачей. Москва: Медицина; 1989.Berenbein BA, Studnitsin AA. (eds.) Differential diagnosis of skin diseases. Guide for doctors. Moscow: Medicine; 1989. (In Russ.)Beales IL. Malignant atrophic papulosis presenting as gastroparesis. Am J Gastroenterol. 2001;96(12):3462. https://doi.org/10.1111/j.1572-0241.2001.05358.xBeales IL. Malignant atrophic papulosis presenting as gastroparesis. Am J Gastroenterol. 2001;96(12):3462. https://doi.org/10.1111/j.1572-0241.2001.05358.xBurg G, Vieluf D, Stolz W, Landthaler M, Braun-Falco O. Malignant atrophic papulosis (Köhlmeier–Degos disease). Hautarzt. 1989;40(8):480–485.Burg G, Vieluf D, Stolz W, Landthaler M, Braun-Falco O. Malignant atrophic papulosis (Köhlmeier–Degos disease). Hautarzt. 1989;40(8):480–485.Gmuca S, Boos MD, Treece A, Narula S, Billinghurst L, Bhatti T et al. Degos disease mimicking primary vasculitis of the CNS. Neurol Neuroimmunol Neuroinfl amm. 2016;3(2):e206. https:// doi.org/10.1212/NXI.0000000000000206Gmuca S, Boos MD, Treece A, Narula S, Billinghurst L, Bhatti T et al. Degos disease mimicking primary vasculitis of the CNS. Neurol Neuroimmunol Neuroinfl amm. 2016;3(2):e206. https:// doi.org/10.1212/NXI.0000000000000206Gutiérrez-Pascual M, Hernández-Martín A, Colmenero I, García-Peñas JJ, López-Pino MA, Torrelo A. Malignant Atrophic Papulosis: A Case Report with Severe Visual and Neurological Impairment. Pediatr Dermatol. 2011;28(3):302–305. https://doi.org/10.1111/j.1525-1470.2010.01287.xGutiérrez-Pascual M, Hernández-Martín A, Colmenero I, García-Peñas JJ, López-Pino MA, Torrelo A. Malignant Atrophic Papulosis: A Case Report with Severe Visual and Neurological Impairment. Pediatr Dermatol. 2011;28(3):302–305. https://doi.org/10.1111/j.1525-1470.2010.01287.xAmato C, Ferri R, Elia M, Cosentino F, Schepis C, Siragusa M, Moschini M. Nervous System Involvement in Degos Disease. Am J Neuroradiol. 2005;26(3):646–649.Amato C, Ferri R, Elia M, Cosentino F, Schepis C, Siragusa M, Moschini M. Nervous System Involvement in Degos Disease. Am J Neuroradiol. 2005;26(3):646–649.Subbiah P, Wijdicks E, Muenter M, Carter J, Connolly S. Skin lesion with a fatal neurologic outcome (Degos’ disease). Neurology. 1996;46(3):636–640. https://doi.org/10.1212/WNL.46.3.636Subbiah P, Wijdicks E, Muenter M, Carter J, Connolly S. Skin lesion with a fatal neurologic outcome (Degos’ disease). Neurology. 1996;46(3):636–640. https://doi.org/10.1212/WNL.46.3.636Matsuura F, Makino K, Fukushima T, Matsubara N, Shibuya M, Higuchi T et al. Optic nerve and spinal cord manifestations of malignant atrophic papulosis (Degos disease). J Neurol Neurosurg Psychiatry. 2006;77(2):260–262. https://doi.org/10.1136/jnnp.2005.066134Matsuura F, Makino K, Fukushima T, Matsubara N, Shibuya M, Higuchi T et al. Optic nerve and spinal cord manifestations of malignant atrophic papulosis (Degos disease). J Neurol Neurosurg Psychiatry. 2006;77(2):260–262. https://doi.org/10.1136/jnnp.2005.066134Oliver B, Boehm M, Rosing DR, Shapiro LS, Dempsey DT, Merkel PA et al. Diffuse atrophic papules and plaques, intermittent abdominal pain, paresthesias, and cardiac abnormalities in a 55-year-old woman. J Am Acad Dermatol. 2016;75(6):1274–1277. https://doi.org/10.1016/j.jaad.2016.09.015Oliver B, Boehm M, Rosing DR, Shapiro LS, Dempsey DT, Merkel PA et al. Diffuse atrophic papules and plaques, intermittent abdominal pain, paresthesias, and cardiac abnormalities in a 55-year-old woman. J Am Acad Dermatol. 2016;75(6):1274–1277. https://doi.org/10.1016/j.jaad.2016.09.015

The authors declare that there are no conflicts of interest present.