vspВопросы современной педиатрииCurrent Pediatrics1682-55271682-5535Издательство «ПедиатрЪ»10.15690/vsp.v23i6.2823vsp-3655Research ArticleКЛИНИЧЕСКОЕ НАБЛЮДЕНИЕCLINICAL OBSERVATIONSПоздняя диагностика системной склеродермии с сосудистыми осложнениями: клинический случайLate Diagnosis of Systemic Scleroderma with Vascular Events: Clinical Casehttps://orcid.org/0009-0000-8825-9815БогомоловаЮ. С.BogomolovaJulia S.

Богомолова Юлия Сергеевна - ассистент кафедры факультетской педиатрии 

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Julikvor26@bk.ruhttps://orcid.org/0000-0002-6078-2361СанталоваГ. В.SantalovaGalina V.

Самара

Samara

ФГБОУ ВО «Самарский государственный медицинский университет» Минздрава РоссииРоссияSamara State Medical UniversityRussian Federation202403012025236495502Copyright © Богомолова Ю.С., Санталова Г.В., 20242024Богомолова Ю.С., Санталова Г.В.Bogomolova J.S., Santalova G.V.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://vsp.spr-journal.ru/jour/article/view/3655

Обоснование. Своевременная диагностика системной склеродермии (ССД) позволяет добиться снижения риска развития осложнений заболевания и инвалидизации пациентов. Однако установление правильного диагноза затрудняет разнообразие клинических форм и вариантов заболевания. Описание клинического случая. ССД у мальчика в возрасте до 5 лет расценивалась как врожденная гемиатрофия кожи, по поводу которой ребенок наблюдался у дерматолога, лечение без эффекта. После установления диагноза ССД в 5-летнем возрасте проводилась терапия метотрексатом и преднизолоном. В возрасте 10 лет появились и в дальнейшем прогрессировали подногтевые язвы и признаки остеолиза дистальных фаланг пальцев. По этому поводу амбулаторно ребенку проводили лечение онихомикоза без эффекта. Ребенок госпитализирован в стационар. В течение 21 сут проводилась вазотропная терапия (пентоксифиллин, нифедипин, алпростадил) с положительным эффектом (отмечено уменьшение площади и глубины подногтевых язв). Заключение. Поздняя диагностика ССД стала причиной развития сосудистых нарушений, а неверная интерпретация клинических признаков этих осложнений привела к их прогрессированию. Вазотропная терапия позволила добиться положительного эффекта.

Background. Timely diagnosis of systemic scleroderma (SS) can reduce the risk of complications and disability in patients. However, correct diagnosis is complicated by the diversity of disease clinical forms and variants. Clinical case description. SS in a boy under 5 years old was considered as congenital skin hemiatrophy, thus he was observed by dermatologist (treatment without effect). Methotrexate and prednisolone therapy was administered at the age of 5 years after diagnosing the SS. Subungual ulcers and distal phalanges osteolysis appeared and further progressed at the age of 10 years. In this regard the child was treated for onychomycosis outpatiently, however with no effect. Later the child was hospitalized. Vasotropic therapy (pentoxifylline, nifedipine, alprostadil) was carried out for 21 days with positive effect: decrease in the area and depth of subungual ulcers. Conclusion. Late diagnosis of SS has led to vascular events whereas incorrect interpretation of clinical signs led to their progression. Vasotropic therapy has achieved positive effect.

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