Синдром Лайєлла: випадок із практики

Abstract

У роботі представлена стисла історична довідка, етіологія та патогенез, сучасна класифікація, клініко-морфологічні зміни та патогістологія ССД-ТЕН. Також наведений випадок із власної практики з описанням деяких клінічних даних та патологоанатомічних змін, виявлених на автопсії жінки 29 років, яка страждала на синдром Лайєлла причиною якого було неконтрольоване вживання нестероїдних протизапальних засобів; The paper considers brief historical background, etiology and pathogenesis, current classification, clinicopathologic changes and histopathology of Stevens-Johnson syndrome – toxic epidermal necrolysis (SJS- TEN). Lyell’s syndrome (toxic epidermal necrolysis) is a rare acute dermatovisceral immunoallergic toxic-induced pathology, characterized by intensive necrosis and detachment of the epidermis with formation of significant blisters and erosions, affecting skin and mucous membranes. Current concepts of SJS and TEN are considered as variants of the same pathological lesion, overlapping into Stevens-Johnson syndrome, also known as toxic epidermal necrolysis (SJS- TEN). Three forms of lesion have been defined depending on the area of epidermis detachment: 1. Stevens-Johnson syndrome (“light form” of TEN) –– detachment of epidermis on more than 10% of body surface area. 2. Transitional form of SJS- TEN (SJS––TEN “overlapping”) –– detachment of epidermis on between10% and 30% of body surface area. 3. TEN (Lyell’s syndrome) – detachment of epidermis on more than 30% of body surface area. Lyell’s syndrome is developed against a background of hypersensitivity of the organism as the reaction to additive effect of toxic, medicinal, infectious agents, plant and animal antigens which are of different effect in different age groups. Immune complex reaction of Type III hypersensitivity is considered to be the leading factor in development of TEN. Pathognomonically, TEN is characterized by necrosis of the epidermis with its further detachment from dermis. TEN affects mucous membranes, such as oral mucosa in the form of aphthous stomatitis, which is further develops in ulcero-necrotic one; genital mucosa (vulvitis, balanoposthitis); ocular mucosa with the development of hemorrhagic and then ulcero-necrotic conjunctivitis. Deep ulcero-necrotic changes and visceral mucosae detachments are also possible. Kidneys are involved in TEN in the form of tubular necrosis which leads to acute renal failure. The loss of fluid, electrolytes and protein through erosive surfaces lead to imbalance of water-salt metabolism, development of infectious complications (often to pneumonia, secondary skin infection) and sepsis that often result in fatal outcome. In the area of blister, generally subepidermal one, necrobiotic changes in epidermis in the form of necrolysis are detected. System of epidermal layers is destroyed, intercellular unions are damaged, hydropic dystrophy of individual epidermal cells, which nuclei are mostly in condition of pycnosis, is observed. Numerous neutrophilic granulocytes are observed between destructive cells. Blister is filled with ballooning cells, lymphocytes and neutrophilic granulocytes. Skin undergoes loosening of collagen and elastic fibers, caused by the oedema and minor perivascular lymphoid infiltrates. Vessels undergo endothelial oedema, and are surrounded by lymphoid infiltrates. Two cases from the own clinical experience have been described, presenting some clinical evidence and pathologicoanatomic changes, detected during autopsy of woman and man, suffered from Lyell’s syndrome, cased by the uncontrolled taking of nonsteroidal anti-inflammatory drugs and complex influence of plant and animal antigens; В работе представлена краткая историческая справка, этиология и патогенез, современная классификация, клинико-морфологические изменения и патогистология ССД-ТэН. Также приведены два случая из собственной практики с описанием некоторых клинических данных и патологоанатомических из- менений, выявленных на аутопсии женщины и мужчины, которые страдали синдромом Лайелла, причинами развития которого было неконтролируемое употребление нестероидных противовоспалительных препара- тов и комплексное влияние растительных и животных антигенов.